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Details on Person Diagram of Bile acid and bile salt metabolism, Defective ABCB11 causes PFIC2 and BRIC2, and Defective CYP27A1 causes CTX
| Class:Id | PathwayDiagram:532427 |
|---|---|
| _displayName | Diagram of Bile acid and bile salt metabolism, Defective ABCB11 causes PFIC2 and BRIC2, and Defective CYP27A1 causes CTX |
| _timestamp | 2026-05-04 14:25:31 |
| created | [InstanceEdit:532428] D'Eustachio, P, 2010-02-26 |
| height | 1760 |
| modified | [InstanceEdit:1679954] Wu, G, 2011-10-20 [InstanceEdit:1878006] Rothfels, K, 2011-10-31 [InstanceEdit:2603292] D'Eustachio, P, 2012-11-19 [InstanceEdit:3229261] Gillespie, ME, 2013-03-26 [InstanceEdit:3907269] Gillespie, ME, 2013-07-18 [InstanceEdit:5340615] Jassal, B, 2014-03-20 [InstanceEdit:5607772] Jassal, Bijay, 2014-07-14 [InstanceEdit:5607773] Jassal, Bijay, 2014-07-14 [InstanceEdit:5637567] Matthews, Lisa, 2014-11-11 [InstanceEdit:5676072] Jassal, Bijay, 2015-02-19 [InstanceEdit:5678573] Jassal, Bijay, 2015-02-24 [InstanceEdit:5684731] D'Eustachio, Peter, 2015-03-21 [InstanceEdit:6788561] Jassal, Bijay, 2015-07-23 [InstanceEdit:6792750] Jassal, Bijay, 2015-08-27 [InstanceEdit:8877143] Jassal, Bijay, 2016-06-21 [InstanceEdit:8878415] Jassal, Bijay, 2016-07-04 [InstanceEdit:8878418] Jassal, Bijay, 2016-07-04 [InstanceEdit:9032311] D'Eustachio, Peter, 2017-12-09 [InstanceEdit:9617029] Jassal, Bijay, 2018-08-16 [InstanceEdit:9652827] Jassal, Bijay, 2019-07-08 [InstanceEdit:9667251] Jassal, Bijay, 2019-11-08 [InstanceEdit:9694015] Jassal, Bijay, 2020-07-08 [InstanceEdit:9697722] Jassal, Bijay, 2020-08-20 [InstanceEdit:9713162] Jassal, Bijay, 2021-01-21 [InstanceEdit:9716699] Jassal, Bijay, 2021-02-24 [InstanceEdit:9716704] Jassal, Bijay, 2021-02-24 [InstanceEdit:9733551] Jassal, Bijay, 2021-06-07 [InstanceEdit:9733974] Jassal, Bijay, 2021-06-14 [InstanceEdit:9733997] Jassal, Bijay, 2021-06-14 [InstanceEdit:9733999] Jassal, Bijay, 2021-06-14 [InstanceEdit:9734003] Jassal, Bijay, 2021-06-14 [InstanceEdit:9734005] Jassal, Bijay, 2021-06-14 [InstanceEdit:9734006] Jassal, Bijay, 2021-06-14 [InstanceEdit:9734007] Jassal, Bijay, 2021-06-14 [InstanceEdit:9734251] Jassal, Bijay, 2021-06-16 [InstanceEdit:9751093] Jassal, Bijay, 2021-08-18 [InstanceEdit:9751103] Jassal, Bijay, 2021-08-18 [InstanceEdit:9758371] Matthews, Lisa, 2021-11-11 [InstanceEdit:9758393] D'Eustachio, Peter, 2021-11-11 [InstanceEdit:9758395] D'Eustachio, Peter, 2021-11-11 [InstanceEdit:9758396] D'Eustachio, Peter, 2021-11-11 [InstanceEdit:9908509] D'Eustachio, Peter, 2024-04-22 [InstanceEdit:9917216] D'Eustachio, Peter, 2024-07-31 [InstanceEdit:9948408] D'Eustachio, Peter, 2025-05-20 [InstanceEdit:9954124] D'Eustachio, Peter, 2025-06-06 [InstanceEdit:9957263] D'Eustachio, Peter, 2025-06-17 [InstanceEdit:9958570] D'Eustachio, Peter, 2025-06-23 [InstanceEdit:9960585] D'Eustachio, Peter, 2025-07-15 [InstanceEdit:9960864] D'Eustachio, Peter, 2025-07-17 [InstanceEdit:9961097] D'Eustachio, Peter, 2025-07-18 [InstanceEdit:9987445] D'Eustachio, Peter, 2026-04-15 [InstanceEdit:9987673] D'Eustachio, Peter, 2026-04-23 [InstanceEdit:9987742] D'Eustachio, Peter, 2026-04-27 [InstanceEdit:9987887] D'Eustachio, Peter, 2026-04-28 [InstanceEdit:9987901] D'Eustachio, Peter, 2026-04-29 [InstanceEdit:9988142] D'Eustachio, Peter, 2026-05-04 |
| representedPathway | [Pathway:194068] Bile acid and bile salt metabolism [Homo sapiens] [Pathway:5678520] Defective ABCB11 causes PFIC2 and BRIC2 [Homo sapiens] [Pathway:5578996] Defective CYP27A1 causes CTX [Homo sapiens] |
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125 73" textPosition="3956 1122"> <Properties> <displayName>3a,7a,12a-trihydroxy-5b-cholest-24-one-CoA</displayName> </Properties> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="186" reactomeId="193465" position="3963 1059" bounds="3946 1044 34 31" textPosition="3949 1048" /> <org.gk.render.RenderableChemical id="188" reactomeId="193403" position="4025 997" bounds="3952 967 147 60" textPosition="3957 979"> <Properties> <displayName>3a,7a-dihydroxy-5b-cholest-24-one-CoA</displayName> </Properties> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="194" reactomeId="193514" position="4067 1057" bounds="4038 1042 59 31" textPosition="4041 1046" /> <org.gk.render.RenderableChemical id="195" reactomeId="192356" position="4263 1141" bounds="4222 1126 83 31" textPosition="4225 1130"> <Properties> <displayName>choloyl-CoA</displayName> </Properties> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="196" 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reactomeId="192067" points="947 1108, 995 1108, 1013 1109" position="995 1108" lineWidth="1.0"> <Inputs> <Input id="38" points="778 1136" /> <Input id="50" points="929 1063" /> <Input id="52" points="887 1063" /> </Inputs> <Outputs> <Output id="47" points="1066 1107" /> <Output id="54" points="1072 1061" /> </Outputs> <Catalysts> <Catalyst id="48" points="993 1059" /> </Catalysts> <Properties> <displayName>4-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholesten-7alpha, 12alpha-diol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="56" reactomeId="192033" points="943 986, 992 985, 1016 986" position="992 985" lineWidth="1.0"> <Inputs> <Input id="31" points="747 968" /> <Input id="50" points="929 1026" /> <Input id="52" points="888 1026" /> </Inputs> <Outputs> <Output id="57" points="1072 975" /> <Output id="54" points="1069 1024" /> </Outputs> <Catalysts> <Catalyst id="48" points="992 1030" /> </Catalysts> <Properties> <displayName>4-cholesten-7alpha-ol-3-one is reduced to 5beta-cholestan-7alpha-ol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="58" reactomeId="192036" points="1218 1106, 1273 1105, 1309 1105" position="1273 1105" lineWidth="1.0"> <Inputs> <Input id="47" points="1189 1106" /> <Input id="62" points="1207 1064" /> <Input id="64" points="1168 1063" /> </Inputs> <Outputs> <Output id="59" points="1331 1116" /> <Output id="66" points="1347 1066" /> </Outputs> <Catalysts> <Catalyst id="670" points="1271 1071" /> </Catalysts> <Properties> <displayName>5Beta-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha, 12alpha-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="67" reactomeId="192160" points="1208 985, 1266 985, 1311 985" position="1266 985" lineWidth="1.0"> <Inputs> <Input id="57" points="1191 979" /> <Input id="62" points="1205 1027" /> <Input id="64" points="1166 1026" /> </Inputs> <Outputs> <Output id="68" points="1339 967" /> <Output id="66" points="1348 1029" /> </Outputs> <Catalysts> <Catalyst id="670" points="1268 1013" /> </Catalysts> <Properties> <displayName>5beta-cholestan-7alpha-ol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha-diol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="70" reactomeId="192010" points="1441 1147, 1463 1147, 1501 1148" position="1463 1147" lineWidth="1.0"> <Inputs> <Input id="59" /> </Inputs> <Outputs> <Output id="71" /> </Outputs> <Catalysts> <Catalyst id="2900" points="1461 1066" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha, 7alpha, 12alpha-triol is translocated from the cytosol to the mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="73" reactomeId="193537" points="1435 941, 1461 942, 1498 940" position="1461 942" lineWidth="1.0"> <Inputs> <Input id="68" /> </Inputs> <Outputs> <Output id="74" /> </Outputs> <Catalysts> <Catalyst id="2900" points="1461 1007" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha, 7alpha-diol is translocated from the cytosol to the mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="75" reactomeId="191999" points="1632 1117, 1687 1117, 1715 1117" position="1687 1117" lineWidth="1.0"> <Inputs> <Input id="76" points="1575 1049" /> <Input id="77" points="1617 1047" /> <Input id="78" points="1539 1043" /> <Input id="71" points="1610 1126" /> </Inputs> <Outputs> <Output id="79" points="1806 1114" /> <Output id="80" points="1724 1085" /> <Output id="81" points="1769 1068" /> </Outputs> <Catalysts> <Catalyst id="82" points="1685 1040" /> </Catalysts> <Properties> <displayName>CYP27A1 27-hydroxylates 5bCHOL3a,7a,12a-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="84" reactomeId="193393" points="1615 978, 1683 977, 1714 976" position="1683 977" lineWidth="1.0"> <Inputs> <Input id="74" points="1594 965" /> <Input id="76" points="1578 1012" /> <Input id="77" points="1615 1010" /> <Input id="78" points="1539 1020" /> </Inputs> <Outputs> <Output id="85" points="1795 976" /> <Output id="80" points="1726 1048" /> <Output id="81" points="1770 1031" /> </Outputs> <Catalysts> <Catalyst id="82" points="1684 1011" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha, 7alpha-diol is hydroxylated to 5beta-cholestan-3alpha, 7alpha, 26-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="88" reactomeId="192042" points="1955 1108, 1990 1108, 2069 1108" position="1990 1108" lineWidth="1.0"> <Inputs> <Input id="79" points="1942 1108" /> <Input id="100" points="1920 1062" /> </Inputs> <Outputs> <Output id="90" points="2097 1110" /> <Output id="95" points="2067 1081" /> <Output id="97" points="2104 1067" /> </Outputs> <Catalysts> <Catalyst id="98" points="1989 1058" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="101" reactomeId="193497" points="1952 990, 1989 991, 2062 990" position="1989 991" lineWidth="1.0"> <Inputs> <Input id="85" points="1926 985" /> <Input id="100" points="1922 1025" /> </Inputs> <Outputs> <Output id="102" points="2096 988" /> <Output id="95" points="2065 1044" /> <Output id="97" points="2102 1030" /> </Outputs> <Catalysts> <Catalyst id="98" points="1989 1029" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha, 7alpha, 26-triol is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="105" reactomeId="192054" points="2261 1115, 2347 1114, 2397 1113" position="2347 1114" lineWidth="1.0"> <Inputs> <Input id="90" points="2236 1115" /> <Input id="114" points="2189 1056" /> <Input id="116" points="2226 1063" /> <Input id="118" points="2267 1065" /> </Inputs> <Outputs> <Output id="106" points="2462 1061" /> <Output id="107" points="2441 1111" /> <Output id="110" points="2415 1061" /> </Outputs> <Catalysts> <Catalyst id="112" points="2347 1059" /> </Catalysts> <Properties> <displayName>3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate (THCA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="120" reactomeId="193460" points="2252 988, 2350 988, 2390 989" position="2350 988" lineWidth="1.0"> <Inputs> <Input id="102" points="2218 987" /> <Input id="114" points="2189 1031" /> <Input id="116" points="2226 1026" /> <Input id="118" points="2264 1028" /> </Inputs> <Outputs> <Output id="121" points="2441 991" /> <Output id="110" points="2411 1024" /> <Output id="106" points="2457 1029" /> </Outputs> <Catalysts> <Catalyst id="112" points="2348 1030" /> </Catalysts> <Properties> <displayName>3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestanoate (DHCA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="123" reactomeId="191971" points="2497 1109, 2515 1109, 2534 1109" position="2515 1109" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="107" /> </Inputs> <Outputs> <Output id="124" /> </Outputs> <Properties> <displayName>THCA is translocated from the mitochondrial matrix to the cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="125" reactomeId="193519" points="2498 995, 2512 997, 2533 997" position="2512 997" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="121" /> </Inputs> <Outputs> <Output id="126" /> </Outputs> <Properties> <displayName>DHCA is translocated from the mitochondrial matrix to the cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="129" reactomeId="193401" points="2648 1183, 2703 1182, 2787 1181" position="2703 1182" lineWidth="1.0"> <Inputs> <Input id="124" points="2580 1130" /> <Input id="130" points="2565 1073" /> <Input id="131" points="2631 1076" /> </Inputs> <Outputs> <Output id="134" points="2913 1132" /> <Output id="135" points="2851 1081" /> <Output id="132" points="2927 1073" /> </Outputs> <Catalysts> <Catalyst id="136" points="2700 1117" /> </Catalysts> <Properties> <displayName>THCA is conjugated with Coenzyme A (SLC27A2 VLCS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="137" reactomeId="193424" points="2634 948, 2696 948, 2791 947" position="2696 948" lineWidth="1.0"> <Inputs> <Input id="126" points="2583 980" /> <Input id="131" points="2629 1039" /> <Input id="130" points="2565 1036" /> </Inputs> <Outputs> <Output id="135" points="2851 1044" /> <Output id="132" points="2927 1048" /> <Output id="140" points="2910 986" /> </Outputs> <Catalysts> <Catalyst id="136" points="2699 1088" /> </Catalysts> <Properties> <displayName>DHCA is conjugated with Coenzyme A (SLC27A2 VLCS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="143" reactomeId="192137" points="2652 968, 2750 967, 2789 966" position="2750 967" lineWidth="1.0"> <Inputs> <Input id="124" points="2571 1093" /> <Input id="131" points="2633 1039" /> <Input id="130" points="2571 1036" /> </Inputs> <Outputs> <Output id="134" points="2933 1095" /> <Output id="135" points="2849 1044" /> <Output id="132" points="2927 1050" /> </Outputs> <Catalysts> <Catalyst id="146" points="2797 1048" /> </Catalysts> <Properties> <displayName>THCA is conjugated with Coenzyme A (SLC27A5 BACS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="147" reactomeId="193407" points="2655 1152, 2757 1153, 2788 1153" position="2757 1153" lineWidth="1.0"> <Inputs> <Input id="131" points="2633 1076" /> <Input id="126" points="2570 1017" /> <Input id="130" points="2569 1073" /> </Inputs> <Outputs> <Output id="135" points="2848 1081" /> <Output id="132" points="2927 1070" /> <Output id="140" points="2937 1023" /> </Outputs> <Catalysts> <Catalyst id="146" points="2798 1077" /> </Catalysts> <Properties> <displayName>DHCA is conjugated with Coenzyme A (SLC27A5 BACS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="152" reactomeId="192325" points="3046 1111, 3070 1111, 3093 1123" position="3070 1111" lineWidth="1.0" reactionType="Transition"> <Inputs> <Input id="134" points="3011 1111" /> <Input id="2926" points="3037 1083" /> <Input id="2927" points="3004 1079" /> </Inputs> <Outputs> <Output id="153" points="3123 1134" /> <Output id="2929" points="3029 1144" /> <Output id="2931" points="3040 1204" /> <Output id="2933" points="3036 1174" /> </Outputs> <Catalysts> <Catalyst id="2924" points="3080 1085" /> </Catalysts> <Properties> <displayName>25(R) THCA-CoA is translocated from the cytosol to the peroxisome</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="154" reactomeId="193482" points="3035 1005, 3069 1006, 3092 993" position="3069 1006" lineWidth="1.0" reactionType="Transition"> <Inputs> <Input id="140" points="3015 1004" /> <Input id="2926" points="3033 1046" /> <Input id="2927" points="2999 1042" /> </Inputs> <Outputs> <Output id="155" points="3125 955" /> <Output id="2955" points="3028 975" /> <Output id="2951" points="3029 943" /> <Output id="2954" points="3037 920" /> </Outputs> <Catalysts> <Catalyst id="2924" points="3081 1043" /> </Catalysts> <Properties> <displayName>25(R) DHCA-CoA is translocated from the cytosol to the peroxisome</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="157" reactomeId="192056" points="3183 1134, 3225 1110, 3280 1133" position="3225 1110" lineWidth="1.0"> <Inputs> <Input id="153" /> </Inputs> <Outputs> <Output id="158" /> </Outputs> <Catalysts> <Catalyst id="159" points="3225 867" /> </Catalysts> <Properties> <displayName>Isomerization of 25(R) THCA-CoA to 25(S) THCA-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="161" reactomeId="193452" points="3159 955, 3201 981, 3250 978" position="3201 981" lineWidth="1.0"> <Inputs> <Input id="155" /> </Inputs> <Outputs> <Output id="162" /> </Outputs> <Catalysts> <Catalyst id="159" points="3223 867" /> </Catalysts> <Properties> <displayName>Isomerization of 25(R) DHCA-CoA to 25(S) DHCA-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="163" reactomeId="192335" points="3347 1114, 3399 1114, 3458 1114" position="3399 1114" lineWidth="1.0"> <Inputs> <Input id="164" points="3316 1078" /> <Input id="158" points="3327 1133" /> </Inputs> <Outputs> <Output id="165" points="3482 1079" /> <Output id="166" points="3512 1115" /> </Outputs> <Catalysts> <Catalyst id="167" points="3399 1074" /> </Catalysts> <Properties> <displayName>25(S) THCA-CoA is dehydrogenated to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="169" reactomeId="193369" points="3361 1012, 3403 1012, 3456 1013" position="3403 1012" lineWidth="1.0"> <Inputs> <Input id="162" points="3331 995" /> <Input id="164" points="3325 1044" /> </Inputs> <Outputs> <Output id="165" points="3480 1042" /> <Output id="170" points="3486 997" /> </Outputs> <Catalysts> <Catalyst id="167" points="3400 1045" /> </Catalysts> <Properties> <displayName>25(S) DHCA-CoA is dehydrogenated to 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="172" reactomeId="192331" points="3630 1111, 3654 1111, 3703 1133" position="3654 1111" lineWidth="1.0"> <Inputs> <Input id="173" points="3616 1079" /> <Input id="166" points="3595 1113" /> </Inputs> <Outputs> <Output id="174" /> </Outputs> <Catalysts> <Catalyst id="175" points="3686 1077" /> </Catalysts> <Properties> <displayName>3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="177" reactomeId="193535" points="3630 1011, 3654 1012, 3715 995" position="3654 1012" lineWidth="1.0"> <Inputs> <Input id="170" points="3592 997" /> <Input id="173" points="3617 1042" /> </Inputs> <Outputs> <Output id="1209" /> </Outputs> <Catalysts> <Catalyst id="175" points="3687 1048" /> </Catalysts> <Properties> <displayName>25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="182" reactomeId="193455" points="3863 1114, 3894 1111, 3911 1110" position="3894 1111" lineWidth="1.0"> <Inputs> <Input id="183" points="3847 1079" /> <Input id="174" points="3844 1126" /> </Inputs> <Outputs> <Output id="184" points="3911 1078" /> <Output id="185" points="3944 1125" /> <Output id="186" points="3947 1078" /> </Outputs> <Catalysts> <Catalyst id="175" points="3743 1077" /> </Catalysts> <Properties> <displayName>(24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="187" reactomeId="193508" points="3855 993, 3894 1007, 3919 1020" position="3894 1007" lineWidth="1.0"> <Inputs> <Input id="183" points="3844 1042" /> <Input id="1209" points="3838 989" /> </Inputs> <Outputs> <Output id="184" points="3914 1041" /> <Output id="186" points="3945 1041" /> <Output id="188" points="3949 1012" /> </Outputs> <Catalysts> <Catalyst id="175" points="3738 1048" /> </Catalysts> <Properties> <displayName>(24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="193" reactomeId="192341" points="4095 1114, 4142 1112, 4181 1111" position="4142 1112" lineWidth="1.0"> <Inputs> <Input id="185" points="4075 1124" /> <Input id="194" points="4074 1076" /> </Inputs> <Outputs> <Output id="195" points="4220 1123" /> <Output id="196" points="4215 1076" /> </Outputs> <Catalysts> <Catalyst id="197" points="4140 1072" /> </Catalysts> <Properties> <displayName>Thiolysis of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA yields choloyl-CoA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="198" reactomeId="193533" points="4110 1013, 4140 1012, 4176 1012" position="4140 1012" lineWidth="1.0"> <Inputs> <Input id="194" points="4082 1039" /> <Input id="188" points="4102 1010" /> </Inputs> <Outputs> <Output id="199" points="4208 999" /> <Output id="196" points="4211 1039" /> </Outputs> <Catalysts> <Catalyst id="197" points="4140 1043" /> </Catalysts> <Properties> <displayName>Thiolysis of 3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA yields chenodeoxycholoyl-CoA (3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="200" reactomeId="193385" points="4433 1192, 4468 1192, 4505 1192" position="4468 1192" lineWidth="1.0"> <Inputs> <Input id="201" points="4394 1206" /> <Input id="195" points="4308 1153" /> </Inputs> <Outputs> <Output id="202" points="4529 1204" /> <Output id="205" points="4530 1169" /> <Output id="2991" points="4576 1181" /> </Outputs> <Catalysts> <Catalyst id="203" points="4467 1160" /> </Catalysts> <Properties> <displayName>Hydrolysis of choloyl-CoA to cholate and CoASH</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="209" reactomeId="193399" points="4529 1226, 4471 1245, 4471 1278" position="4471 1245" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="202" /> </Inputs> <Outputs> <Output id="210" /> </Outputs> <Properties> <displayName>Cholate is translocated from the peroxisomal matrix to the cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="212" reactomeId="192312" points="4312 1108, 4395 1108, 4432 1108" position="4395 1108" lineWidth="1.0"> <Inputs> <Input id="213" points="4318 1075" /> <Input id="195" points="4286 1123" /> </Inputs> <Outputs> <Output id="214" points="4468 1108" /> <Output id="222" points="4486 1076" /> <Output id="2995" points="4445 1073" /> </Outputs> <Catalysts> <Catalyst id="215" points="4394 1072" /> </Catalysts> <Properties> <displayName>Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholate</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="218" reactomeId="193491" points="4309 1007, 4392 1008, 4433 1008" position="4392 1008" lineWidth="1.0"> <Inputs> <Input id="213" points="4318 1040" /> <Input id="199" points="4290 999" /> </Inputs> <Outputs> <Output id="219" points="4485 990" /> <Output id="2995" points="4444 1036" /> <Output id="222" points="4486 1039" /> </Outputs> <Catalysts> <Catalyst id="215" points="4393 1043" /> </Catalysts> <Properties> <displayName>Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholate</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="223" reactomeId="192315" points="4618 1065, 4648 1065, 4668 1065" position="4648 1065" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="224" /> </Inputs> <Outputs> <Output id="225" /> </Outputs> <Properties> <displayName>Bile salts are translocated from the peroxisomal matrix to the cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="227" reactomeId="193362" points="4762 1041, 4782 1039, 4783 956" position="4782 1039" lineWidth="1.0"> <Inputs> <Input id="647" points="4742 1024" /> <Input id="648" points="4709 1027" /> <Input id="225" points="4731 1048" /> </Inputs> <Outputs> <Output id="230" points="4750 955" /> <Output id="3000" points="4725 925" /> <Output id="229" points="4840 953" /> <Output id="3002" points="4748 905" /> </Outputs> <Catalysts> <Catalyst id="232" points="4781 1089" /> </Catalysts> <Properties> <displayName>ABCB11 transports bile salts from cytosol to extracellular region</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="240" reactomeId="192061" points="266 239, 266 274, 266 295" position="266 274" lineWidth="1.0"> <Inputs> <Input id="245" points="300 201" /> <Input id="247" points="293 229" /> <Input id="249" points="242 205" /> <Input id="251" points="227 227" /> </Inputs> <Outputs> <Output id="241" points="289 303" /> <Output id="242" points="220 320" /> <Output id="253" points="286 286" /> </Outputs> <Catalysts> <Catalyst id="243" points="229 272" /> </Catalysts> <Properties> <displayName>CYP46A1 24-hydroxylates CHOL</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="255" reactomeId="193776" points="242 338, 366 339, 414 339" position="366 339" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="242" /> </Inputs> <Outputs> <Output id="256" /> </Outputs> <Properties> <displayName>Efflux of 24-hydroxycholesterol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="257" reactomeId="193767" points="507 340, 554 341, 580 340, 582 142, 738 141, 738 169, 717 189" position="554 341" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="256" /> </Inputs> <Outputs> <Output id="259" /> </Outputs> <Properties> <displayName>Influx of 24-hydroxycholesterol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="261" reactomeId="192178" points="783 229, 783 264, 783 289" position="783 264" lineWidth="1.0"> <Inputs> <Input id="3005" points="783 195" /> <Input id="259" points="750 218" /> <Input id="270" points="815 218" /> </Inputs> <Outputs> <Output id="3007" points="848 290" /> <Output id="263" points="719 325" /> <Output id="3010" points="813 270" /> <Output id="3012" points="820 314" /> </Outputs> <Catalysts> <Catalyst id="264" points="732 265" /> </Catalysts> <Properties> <displayName>CYP39A1 7-hydroxylates 24OH-CHOL</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="274" reactomeId="193789" points="751 388, 753 409, 753 428" position="753 409" lineWidth="1.0"> <Inputs> <Input id="263" points="713 362" /> <Input id="282" points="770 382" /> </Inputs> <Outputs> <Output id="275" points="724 451" /> <Output id="280" points="770 426" /> <Output id="284" points="780 452" /> </Outputs> <Catalysts> <Catalyst id="277" points="727 408" /> </Catalysts> <Properties> <displayName>Cholest-5-ene-3beta,7alpha,24(S)-triol is oxidized and isomerized to 4-cholesten-7alpha,24(S)-diol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="311" reactomeId="193746" points="912 514, 983 532, 1012 533" position="983 532" lineWidth="1.0"> <Inputs> <Input id="275" points="756 485" /> <Input id="309" points="908 573" /> <Input id="307" points="862 567" /> </Inputs> <Outputs> <Output id="312" points="1048 534" /> <Output id="304" points="1036 570" /> </Outputs> <Catalysts> <Catalyst id="302" points="983 495" /> </Catalysts> <Properties> <displayName>4-cholesten-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-7alpha,24(S)-diol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="326" reactomeId="193758" points="1215 538, 1282 538, 1307 538" position="1282 538" lineWidth="1.0"> <Inputs> <Input id="312" points="1186 537" /> <Input id="320" points="1172 572" /> <Input id="322" points="1209 572" /> </Inputs> <Outputs> <Output id="327" points="1332 510" /> <Output id="324" points="1354 570" /> </Outputs> <Catalysts> <Catalyst id="668" points="1280 558" /> </Catalysts> <Properties> <displayName>5beta-cholestan-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,24(S)-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="331" reactomeId="193715" points="1430 476, 1461 476, 1497 476" position="1461 476" lineWidth="1.0"> <Inputs> <Input id="327" /> </Inputs> <Outputs> <Output id="332" /> </Outputs> <Catalysts> <Catalyst id="2897" points="1461 515" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha,7alpha,24(S)-triol is translocated from the cytosol to the mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="353" reactomeId="193792" points="1625 529, 1698 530, 1744 530" position="1698 530" lineWidth="1.0"> <Inputs> <Input id="332" points="1592 502" /> <Input id="347" points="1619 568" /> <Input id="345" points="1565 595" /> <Input id="343" points="1555 562" /> </Inputs> <Outputs> <Output id="354" points="1773 529" /> <Output id="351" points="1773 594" /> <Output id="340" points="1826 607" /> </Outputs> <Catalysts> <Catalyst id="349" points="1698 572" /> </Catalysts> <Properties> <displayName>CYP27A1 27-hydroxylates 5β-CHOL3α,7α,24(s)-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="371" reactomeId="193719" points="1933 532, 2027 533, 2050 535" position="2027 533" lineWidth="1.0"> <Inputs> <Input id="354" points="1916 531" /> <Input id="364" points="1896 562" /> <Input id="366" points="1919 576" /> <Input id="368" points="1956 602" /> </Inputs> <Outputs> <Output id="373" points="2082 522" /> <Output id="370" points="2090 600" /> <Output id="359" points="2117 571" /> </Outputs> <Catalysts> <Catalyst id="362" points="2027 577" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha,7alpha,24(S),27-tetrol is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="390" reactomeId="193737" points="2232 540, 2355 540, 2389 540" position="2355 540" lineWidth="1.0"> <Inputs> <Input id="373" points="2208 531" /> <Input id="383" points="2291 610" /> <Input id="381" points="2246 601" /> <Input id="379" points="2213 573" /> </Inputs> <Outputs> <Output id="391" points="2408 540" /> <Output id="388" points="2411 608" /> <Output id="376" points="2444 580" /> </Outputs> <Catalysts> <Catalyst id="385" points="2355 577" /> </Catalysts> <Properties> <displayName>3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate (3,7,24THCA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="395" reactomeId="193786" points="2504 540, 2517 540, 2553 508" position="2517 540" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="391" /> </Inputs> <Outputs> <Output id="396" /> </Outputs> <Properties> <displayName>3,7,24THCA is translocated from the mitochondrial matrix to the cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="410" reactomeId="193711" points="2649 701, 2767 701, 2816 701" position="2767 701" lineWidth="1.0"> <Inputs> <Input id="396" points="2574 508" /> <Input id="405" points="2641 608" /> <Input id="399" points="2573 610" /> </Inputs> <Outputs> <Output id="413" points="2902 681" /> <Output id="409" points="2929 597" /> <Output id="407" points="2863 623" /> <Output id="401" points="2828 589" /> </Outputs> <Catalysts> <Catalyst id="403" points="2767 663" /> </Catalysts> <Properties> <displayName>3,7,24THCA is conjugated with Coenzyme A (SLC27A5 BACS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="423" reactomeId="193743" points="2644 466, 2701 464, 2813 462" position="2701 464" lineWidth="1.0"> <Inputs> <Input id="396" points="2617 475" /> <Input id="405" points="2640 571" /> <Input id="399" points="2571 573" /> </Inputs> <Outputs> <Output id="413" points="2941 649" /> <Output id="407" points="2865 586" /> <Output id="409" points="2926 563" /> <Output id="401" points="2827 552" /> </Outputs> <Catalysts> <Catalyst id="420" points="2702 527" /> </Catalysts> <Properties> <displayName>3,7,24THCA is conjugated with Coenzyme A (SLC27A2 VLCS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="429" reactomeId="193753" points="3041 653, 3071 654, 3105 654" position="3071 654" lineWidth="1.0" reactionType="Transition"> <Inputs> <Input id="413" points="3009 660" /> <Input id="2959" points="3005 621" /> <Input id="2958" points="3038 622" /> </Inputs> <Outputs> <Output id="430" points="3245 654" /> <Output id="2971" points="3029 710" /> <Output id="2973" points="3028 748" /> <Output id="2975" points="3031 787" /> </Outputs> <Catalysts> <Catalyst id="2961" points="3083 625" /> </Catalysts> <Properties> <displayName>3,7,24THCA-CoA is translocated from the cytosol to the peroxisome</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="435" reactomeId="193736" points="3274 682, 3262 719, 3316 720, 3294 682" position="3262 719" lineWidth="1.0"> <Inputs> <Input id="430" /> </Inputs> <Outputs> <Output id="430" /> </Outputs> <Catalysts> <Catalyst id="159" points="3229 838" /> </Catalysts> <Properties> <displayName>Isomerization of 3,7,24THCA-CoA to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="444" reactomeId="192123" points="290 1515, 292 1550, 292 1572" position="292 1550" lineWidth="1.0"> <Inputs> <Input id="445" points="254 1506" /> <Input id="451" points="313 1503" /> <Input id="453" points="311 1475" /> <Input id="455" points="272 1474" /> </Inputs> <Outputs> <Output id="446" points="256 1591" /> <Output id="447" points="313 1576" /> <Output id="457" points="314 1562" /> </Outputs> <Catalysts> <Catalyst id="449" points="263 1550" /> </Catalysts> <Properties> <displayName>CYP27A1 27-hydroxylates CHOL</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="458" reactomeId="193812" points="278 1609, 418 1609, 446 1609" position="418 1609" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="446" /> </Inputs> <Outputs> <Output id="459" /> </Outputs> <Properties> <displayName>Efflux of 27-hydroxycholesterol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="460" reactomeId="193801" points="539 1612, 556 1613, 583 1613, 583 1717, 746 1716, 746 1664, 723 1638" position="556 1613" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="459" /> </Inputs> <Outputs> <Output id="461" /> </Outputs> <Properties> <displayName>Influx of 27-hydroxycholesterol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="463" reactomeId="191972" points="813 1605, 811 1567, 811 1536" position="811 1567" lineWidth="1.0"> <Inputs> <Input id="461" points="751 1613" /> <Input id="468" points="799 1629" /> <Input id="471" points="831 1629" /> <Input id="473" points="832 1608" /> </Inputs> <Outputs> <Output id="464" points="755 1523" /> <Output id="465" points="846 1539" /> <Output id="475" points="842 1517" /> </Outputs> <Catalysts> <Catalyst id="663" points="775 1567" /> </Catalysts> <Inhibitors> <Inhibitor id="767" points="978 1568" /> </Inhibitors> <Properties> <displayName>27-hydroxycholesterol is 7alpha-hydroxylated</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="477" reactomeId="193816" points="762 1475, 762 1454, 761 1433" position="762 1454" lineWidth="1.0"> <Inputs> <Input id="464" points="736 1483" /> <Input id="482" points="792 1471" /> </Inputs> <Outputs> <Output id="478" points="725 1404" /> <Output id="484" points="805 1431" /> <Output id="486" points="772 1422" /> </Outputs> <Catalysts> <Catalyst id="480" points="737 1450" /> </Catalysts> <Properties> <displayName>Cholest-5-ene-3beta,7alpha,27-triol is oxidized and isomerized to 4-cholesten-7alpha,27-diol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="488" reactomeId="193845" points="773 1334, 771 1316, 775 1296" position="771 1316" lineWidth="1.0"> <Inputs> <Input id="478" points="727 1367" /> <Input id="497" points="778 1344" /> <Input id="499" points="793 1328" /> <Input id="501" points="805 1345" /> </Inputs> <Outputs> <Output id="1210" points="731 1233" /> <Output id="1211" points="778 1269" /> <Output id="495" points="800 1282" /> </Outputs> <Catalysts> <Catalyst id="2894" points="686 1062, 628 1128, 630 1314" /> </Catalysts> <Properties> <displayName>CYP8B1 12-hydroxylates 4CHOL7a,27DONE</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="506" reactomeId="193821" points="967 1250, 1006 1250, 1047 1251" position="1006 1250" lineWidth="1.0"> <Inputs> <Input id="1210" points="772 1215" /> <Input id="511" points="952 1292" /> <Input id="513" points="913 1292" /> </Inputs> <Outputs> <Output id="507" points="1066 1250" /> <Output id="516" points="1080 1291" /> </Outputs> <Catalysts> <Catalyst id="509" points="1005 1295" /> </Catalysts> <Properties> <displayName>4-cholesten-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-7alpha,12alpha,27-triol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="518" reactomeId="193824" points="962 1380, 1007 1380, 1026 1381" position="1007 1380" lineWidth="1.0"> <Inputs> <Input id="478" points="774 1383" /> <Input id="511" points="950 1329" /> <Input id="513" points="909 1329" /> </Inputs> <Outputs> <Output id="519" points="1053 1381" /> <Output id="516" points="1078 1328" /> </Outputs> <Catalysts> <Catalyst id="509" points="1005 1324" /> </Catalysts> <Properties> <displayName>4-cholesten-7alpha,27-diol-3-one is reduced to 5beta-cholestan-7alpha,27-diol-3-one</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="525" reactomeId="193800" points="1225 1249, 1272 1248, 1290 1248" position="1272 1248" lineWidth="1.0"> <Inputs> <Input id="507" points="1205 1248" /> <Input id="530" points="1210 1292" /> <Input id="532" points="1174 1290" /> </Inputs> <Outputs> <Output id="526" points="1319 1245" /> <Output id="534" points="1340 1287" /> </Outputs> <Catalysts> <Catalyst id="659" points="1272 1279" /> </Catalysts> <Properties> <displayName>5Beta-cholestan-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="535" reactomeId="193841" points="1219 1380, 1272 1380, 1290 1379" position="1272 1380" lineWidth="1.0"> <Inputs> <Input id="519" points="1198 1380" /> <Input id="532" points="1169 1327" /> <Input id="530" points="1208 1329" /> </Inputs> <Outputs> <Output id="536" points="1311 1380" /> <Output id="534" points="1345 1324" /> </Outputs> <Catalysts> <Catalyst id="659" points="1272 1333" /> </Catalysts> <Properties> <displayName>5beta-cholestan-7alpha,27-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,27-triol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="538" reactomeId="193832" points="1444 1243, 1462 1245, 1806 1132" position="1462 1245" lineWidth="1.0"> <Inputs> <Input id="526" /> </Inputs> <Outputs> <Output id="79" /> </Outputs> <Catalysts> <Catalyst id="2903" points="1462 1282" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is translocated from the cytosol to the mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="539" reactomeId="193808" points="1442 1381, 1461 1381, 1651 1354, 1843 1010" position="1461 1381" lineWidth="1.0"> <Inputs> <Input id="536" /> </Inputs> <Outputs> <Output id="85" /> </Outputs> <Catalysts> <Catalyst id="2903" points="1461 1341" /> </Catalysts> <Properties> <displayName>5beta-cholestan-3alpha,7alpha,27-triol is translocated from the cytosol to the mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="542" reactomeId="191983" points="317 961, 317 916, 317 879" position="317 916" lineWidth="1.0"> <Inputs> <Input id="543" points="317 1012" /> <Input id="550" points="337 980" /> <Input id="552" points="336 960" /> <Input id="548" points="347 934" /> </Inputs> <Outputs> <Output id="544" points="296 872" /> <Output id="545" points="306 839" /> <Output id="554" points="342 872" /> </Outputs> <Catalysts> <Catalyst id="546" points="291 916" /> </Catalysts> <Properties> <displayName>Cholesterol is hydroxylated to 25-hydroxycholesterol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="557" reactomeId="192065" points="302 778, 303 745, 302 709" position="303 745" lineWidth="1.0"> <Inputs> <Input id="545" points="302 803" /> <Input id="568" points="356 807" /> <Input id="3025" points="356 779" /> <Input id="3029" points="340 757" /> </Inputs> <Outputs> <Output id="3026" points="351 672" /> <Output id="559" points="302 642" /> <Output id="3031" points="341 701" /> </Outputs> <Catalysts> <Catalyst id="674" points="153 866" /> </Catalysts> <Inhibitors> <Inhibitor id="722" points="191 741" /> </Inhibitors> <Properties> <displayName>CYP7B1 7-hydroxylates 25OH-CHOL</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="578" reactomeId="194187" points="5247 950, 5247 984, 5247 1023" position="5247 984" lineWidth="1.0"> <Inputs> <Input id="580" points="5290 932" /> <Input id="2487" points="5194 933" /> </Inputs> <Outputs> <Output id="583" points="5286 1036" /> <Output id="2224" points="5247 1106" /> </Outputs> <Catalysts> <Catalyst id="813" points="5217 984" /> </Catalysts> <Activators> <Activator id="676" points="5764 1097" /> </Activators> <Properties> <displayName>SLC10A2 transports bile salts and acids and Na+ from extracellular region to cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="597" reactomeId="194121" points="4834 1351, 4781 1351, 4743 1350" position="4781 1351" lineWidth="1.0"> <Inputs> <Input id="2046" points="4925 1374" /> <Input id="601" stoichiometry="2" points="4877 1323" /> </Inputs> <Outputs> <Output id="2891" stoichiometry="2" points="4702 1318" /> <Output id="2893" points="4708 1371" /> </Outputs> <Catalysts> <Catalyst id="599" points="4781 1314" /> </Catalysts> <Properties> <displayName>SLC10A1 co-transport bile salts and Na+ from extracellular region to cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="606" reactomeId="194130" points="4896 1391, 4783 1391, 4733 1392" position="4783 1391" lineWidth="1.0"> <Inputs> <Input id="2046" /> </Inputs> <Outputs> <Output id="2893" /> </Outputs> <Catalysts> <Catalyst id="609" points="4783 1423" /> </Catalysts> <Properties> <displayName>Transport (influx) of bile salts and acids by OATP-A</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="612" reactomeId="194083" points="4853 1639, 4782 1666, 4711 1634" position="4782 1666" lineWidth="1.0"> <Inputs> <Input id="2470" /> </Inputs> <Outputs> <Output id="614" /> </Outputs> <Catalysts> <Catalyst id="615" points="4782 1691" /> </Catalysts> <Properties> <displayName>SLCO1B1 transports ALB:(GCCA,TCCA) from extracellular region to cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="617" reactomeId="194079" points="4844 1604, 4783 1588, 4728 1599" position="4783 1588" lineWidth="1.0"> <Inputs> <Input id="2470" /> </Inputs> <Outputs> <Output id="614" /> </Outputs> <Catalysts> <Catalyst id="618" points="4782 1554" /> </Catalysts> <Properties> <displayName>SLCO1B3 transports ALB:(GCCA, TCCA) from extracellular region to cytosol</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="621" reactomeId="159425" points="4543 1562, 4543 1541, 4543 1506" position="4543 1541" lineWidth="1.0"> <Inputs> <Input id="626" points="4530 1567" /> <Input id="623" points="4544 1616" /> <Input id="628" points="4559 1568" /> </Inputs> <Outputs> <Output id="624" points="4576 1517" /> <Output id="625" points="4543 1462" /> <Output id="632" points="4570 1491" /> <Output id="634" points="4510 1493" /> </Outputs> <Catalysts> <Catalyst id="630" points="4470 1539" /> </Catalysts> <Properties> <displayName>Cytosolic cholate and chenodeoxycholate are conjugated with Coenzyme A (SLC27A5 BACS)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="638" reactomeId="159431" points="4542 1372, 4542 1333, 4542 1292" position="4542 1333" lineWidth="1.0"> <Inputs> <Input id="639" points="4557 1375" /> <Input id="625" points="4542 1399" /> </Inputs> <Outputs> <Output id="642" points="4579 1294" /> <Output id="225" points="4701 1083, 4657 1252" /> </Outputs> <Catalysts> <Catalyst id="640" points="4512 1332" /> </Catalysts> <Properties> <displayName>Cytosolic chenodeoxycholoyl-CoA or choloyl-CoA are conjugated with glycine or taurine</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.EntitySetAndMemberLink id="649" points="4449 1304, 4263 1379, 4263 1626, 4504 1632" position="4263 1502" lineWidth="0.5" lineColor="0 0 0"> <Inputs> <Input id="210" /> </Inputs> <Outputs> <Output id="623" /> </Outputs> </org.gk.render.EntitySetAndMemberLink> <org.gk.render.RenderableReaction id="879" reactomeId="5340195" points="5491 1114, 5491 1113, 5539 1113" position="5491 1113" lineWidth="1.0" reactionType="Association"> <Inputs> <Input id="880" points="5455 1097" /> <Input id="881" points="5457 1141" /> </Inputs> <Outputs> <Output id="882" /> </Outputs> <Catalysts> <Catalyst id="880" points="5455 1084, 5488 1080" /> </Catalysts> <Properties> <displayName>NR1H4 binds DCA, CDCA, LCHA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="887" reactomeId="8873850" points="4232 1696, 4271 1696, 4321 1694" position="4271 1696" lineWidth="1.0" reactionType="Association"> <Inputs> <Input id="889" points="4193 1696" /> <Input id="2921" points="4187 1679" /> </Inputs> <Outputs> <Output id="891" /> </Outputs> <Catalysts> <Catalyst id="889" points="4193 1703, 4270 1726" /> </Catalysts> <Properties> <displayName>STARD5 binds DCA, LCA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="897" reactomeId="8867667" points="307 1070, 335 1070, 378 1070" position="335 1070" lineWidth="1.0"> <Inputs> <Input id="898" /> </Inputs> <Outputs> <Output id="899" /> </Outputs> <Catalysts> <Catalyst id="900" points="334 1096" /> </Catalysts> <Properties> <displayName>OSBPs transport 25OH-CHOL from ER membrane to plasma membrane</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="1202" reactomeId="8868402" points="258 1173, 258 1171, 258 1170" position="258 1171" lineWidth="1.0"> <Inputs> <Input id="1203" points="205 1190" /> <Input id="898" points="259 1089" /> </Inputs> <Outputs> <Output id="1204" points="258 1206" /> <Output id="1205" points="210 1140" /> </Outputs> <Catalysts> <Catalyst id="1206" points="299 1171" /> </Catalysts> <Properties> <displayName>OSBP exchanges 25OH-CHOL with PI4P from ER membrane to Golgi membrane</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.EntitySetAndEntitySetLink id="1212" points="4536 990, 4574 1048" position="4555 1019" lineWidth="0.5" lineColor="128 128 128"> <Inputs> <Input id="219" /> </Inputs> <Outputs> <Output id="224" /> </Outputs> </org.gk.render.EntitySetAndEntitySetLink> <org.gk.render.EntitySetAndEntitySetLink id="1213" points="4564 1083, 4543 1091" position="4553 1087" lineWidth="0.5" lineColor="128 128 128"> <Inputs> <Input id="224" /> </Inputs> <Outputs> <Output id="214" /> </Outputs> </org.gk.render.EntitySetAndEntitySetLink> <org.gk.render.RenderableReaction id="1685" reactomeId="9733973" points="5116 1189, 5118 1189, 5116 1144" position="5118 1189" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="579" points="5033 1137" /> <Input id="2224" points="5225 1141" /> </Inputs> <Outputs> <Output id="582" /> </Outputs> <Properties> <displayName>Bile salts bind FABP6</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="1936" reactomeId="9733969" points="5116 1099, 5116 1055, 5119 1060" position="5116 1055" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="582" /> </Inputs> <Outputs> <Output id="579" points="5035 1108" /> <Output id="2224" points="5224 1106" /> </Outputs> <Properties> <displayName>Bile salts dissociate from FABP6</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2043" reactomeId="9733964" points="5245 1141, 5215 1292, 5203 1329, 5006 1374" position="5215 1292" lineWidth="1.0"> <Inputs> <Input id="2224" /> </Inputs> <Outputs> <Output id="2046" /> </Outputs> <Catalysts> <Catalyst id="2044" points="5174 1292" /> </Catalysts> <Properties> <displayName>SLC51A:SLC51B transports bile salts from cytosol to extracellular region</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2061" reactomeId="194153" points="5248 1204, 5248 1291, 5251 1291" position="5248 1291" lineWidth="1.0"> <Inputs> <Input id="2234" points="5278 1185" /> <Input id="2224" points="5248 1141" /> </Inputs> <Outputs> <Output id="2233" points="5281 1265" /> <Output id="2046" points="5028 1381, 5225 1348" /> <Output id="2066" points="5332 1253" /> </Outputs> <Catalysts> <Catalyst id="2064" points="5316 1291" /> </Catalysts> <Properties> <displayName>ABCC3 transports bile salts from cytosol to extracellular region</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2370" reactomeId="9733960" points="4906 1489, 4867 1489, 4866 1488" position="4867 1489" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="2236" /> </Inputs> <Outputs> <Output id="587" points="4959 1573" /> <Output id="2046" points="4950 1409" /> </Outputs> <Properties> <displayName>Bile salts and acids dissociate from ALB</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.EntitySetAndEntitySetLink id="2471" points="4873 1604, 4825 1489, 4945 1409" position="4825 1489" lineWidth="0.5" lineColor="128 128 128"> <Inputs> <Input id="2470" /> </Inputs> <Outputs> <Output id="2046" /> </Outputs> </org.gk.render.EntitySetAndEntitySetLink> <org.gk.render.EntitySetAndEntitySetLink id="2488" points="4912 948, 5103 922" position="5007 935" lineWidth="0.5" lineColor="128 128 128"> <Inputs> <Input id="229" /> </Inputs> <Outputs> <Output id="2487" /> </Outputs> </org.gk.render.EntitySetAndEntitySetLink> <org.gk.render.RenderableReaction id="2750" reactomeId="9733545" points="5077 1490, 5075 1488, 5027 1488" position="5075 1488" lineWidth="1.0" reactionType="Omitted Process"> <Inputs> <Input id="2046" points="4975 1409" /> <Input id="587" points="4984 1573" /> </Inputs> <Outputs> <Output id="2236" /> </Outputs> <Properties> <displayName>Bile salts and acids bind ALB</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2887" reactomeId="9733975" points="5226 856, 5225 856, 5276 856" position="5225 856" lineWidth="1.0" reactionType="Association"> <Inputs> <Input id="2888" points="5173 809" /> <Input id="2487" points="5180 898" /> </Inputs> <Outputs> <Output id="2889" /> </Outputs> <Properties> <displayName>Bile salts and acids bind bile acid/salt sequestrants</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2919" reactomeId="5340251" points="5683 1111, 5716 1111, 5764 1111" position="5716 1111" lineWidth="1.0"> <Inputs> <Input id="882" points="5666 1111" /> <Input id="2920" points="5687 1143" /> </Inputs> <Outputs> <Output id="676" /> </Outputs> <Catalysts> <Catalyst id="2920" points="5697 1143" /> </Catalysts> <Properties> <displayName>NR1H4:DCA,CDCA,LCHA binds RXRA and NCOA1,2</displayName> </Properties> </org.gk.render.RenderableReaction> </Edges> <Pathways /> </Process> |
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No pathways have been reviewed or authored by Diagram of Bile acid and bile salt metabolism, Defective ABCB11 causes PFIC2 and BRIC2, and Defective CYP27A1 causes CTX (532427)
