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Details on Person Diagram of Branched-chain amino acid catabolism, Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD, Loss-of-function mutations in DBT cause MSUD2, Loss-of-function mutations in DLD cause MSUD3/DLDD, H139Hfs13* PPM1K causes a mild variant of MSUD, Branched-chain ketoacid dehydrogenase kinase deficiency, Isovaleric acidemia, 3-Methylcrotonyl-CoA carboxylase deficiency, 3-methylglutaconic aciduria, Beta-ketothiolase deficiency, Mitochondrial short-chain enoyl-CoA hydratase deficiency 1, and 3-hydroxyisobutyryl-CoA hydrolase deficiency
| Class:Id | PathwayDiagram:6788658 |
|---|---|
| _displayName | Diagram of Branched-chain amino acid catabolism, Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD, Loss-of-function mutations in DBT cause MSUD2, Loss-of-function mutations in DLD cause MSUD3/DLDD, H139Hfs13* PPM1K causes a mild variant of MSUD, Branched-chain ketoacid dehydrogenase kinase deficiency, Isovaleric acidemia, 3-Methylcrotonyl-CoA carboxylase deficiency, 3-methylglutaconic aciduria, Beta-ketothiolase deficiency, Mitochondrial short-chain enoyl-CoA hydratase deficiency 1, and 3-hydroxyisobutyryl-CoA hydrolase deficiency |
| _timestamp | 2026-02-25 09:39:31 |
| created | [InstanceEdit:6788662] Jassal, Bijay, 2015-07-24 |
| height | 1667 |
| modified | [InstanceEdit:8949408] D'Eustachio, Peter, 2016-11-23 [InstanceEdit:8949410] D'Eustachio, Peter, 2016-11-23 [InstanceEdit:8963945] Jassal, Bijay, 2017-02-14 [InstanceEdit:9640340] D'Eustachio, Peter, 2019-03-25 [InstanceEdit:9672777] D'Eustachio, Peter, 2020-01-02 [InstanceEdit:9672919] D'Eustachio, Peter, 2020-01-03 [InstanceEdit:9859160] Stephan, Ralf, 2024-01-16 [InstanceEdit:9859165] Stephan, Ralf, 2024-01-16 [InstanceEdit:9859173] Stephan, Ralf, 2024-01-16 [InstanceEdit:9859247] Stephan, Ralf, 2024-01-17 [InstanceEdit:9859576] Stephan, Ralf, 2024-01-21 [InstanceEdit:9859625] Stephan, Ralf, 2024-01-23 [InstanceEdit:9907327] Stephan, Ralf, 2024-04-06 [InstanceEdit:9907331] Stephan, Ralf, 2024-04-06 [InstanceEdit:9907439] Rothfels, Karen, 2024-04-10 [InstanceEdit:9907442] Rothfels, Karen, 2024-04-10 [InstanceEdit:9907571] Rothfels, Karen, 2024-04-12 [InstanceEdit:9910828] Stephan, Ralf, 2024-05-21 [InstanceEdit:9912486] Rothfels, Karen, 2024-06-08 [InstanceEdit:9912548] Rothfels, Karen, 2024-06-08 [InstanceEdit:9914234] Rothfels, Karen, 2024-06-21 [InstanceEdit:9914277] Rothfels, Karen, 2024-06-23 [InstanceEdit:9914359] Rothfels, Karen, 2024-06-23 [InstanceEdit:9915357] Rothfels, Karen, 2024-07-04 [InstanceEdit:9917131] Stephan, Ralf, 2024-07-30 [InstanceEdit:9917135] Stephan, Ralf, 2024-07-30 [InstanceEdit:9918337] Rothfels, Karen, 2024-08-14 [InstanceEdit:9918347] Rothfels, Karen, 2024-08-14 [InstanceEdit:9918399] Rothfels, Karen, 2024-08-15 [InstanceEdit:9918573] Matthews, Lisa, 2024-08-17 [InstanceEdit:9918918] Rothfels, Karen, 2024-08-18 [InstanceEdit:9918923] Rothfels, Karen, 2024-08-18 [InstanceEdit:9925948] D'Eustachio, Peter, 2024-10-28 [InstanceEdit:9927581] Matthews, Lisa, 2024-11-08 [InstanceEdit:9927582] Matthews, Lisa, 2024-11-08 [InstanceEdit:9928082] D'Eustachio, Peter, 2024-11-11 [InstanceEdit:9928083] D'Eustachio, Peter, 2024-11-11 [InstanceEdit:9983645] Stephan, Ralf, 2026-02-25 |
| representedPathway | [Pathway:70895] Branched-chain amino acid catabolism [Homo sapiens] [Pathway:9865125] Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD [Homo sapiens] [Pathway:9865113] Loss-of-function mutations in DBT cause MSUD2 [Homo sapiens] [Pathway:9907570] Loss-of-function mutations in DLD cause MSUD3/DLDD [Homo sapiens] [Pathway:9912529] H139Hfs13* PPM1K causes a mild variant of MSUD [Homo sapiens] [Pathway:9912481] Branched-chain ketoacid dehydrogenase kinase deficiency [Homo sapiens] [Pathway:9914355] Isovaleric acidemia [Homo sapiens] [Pathway:9909438] 3-Methylcrotonyl-CoA carboxylase deficiency [Homo sapiens] [Pathway:9914274] 3-methylglutaconic aciduria [Homo sapiens] [Pathway:9915355] Beta-ketothiolase deficiency [Homo sapiens] [Pathway:9916720] Mitochondrial short-chain enoyl-CoA hydratase deficiency 1 [Homo sapiens] [Pathway:9916722] 3-hydroxyisobutyryl-CoA hydrolase deficiency [Homo sapiens] |
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<org.gk.render.RenderableChemical id="3027" reactomeId="29374" position="676 628" bounds="645 613 62 31" textPosition="651 617"> <Properties> <displayName>CoA-SH</displayName> </Properties> <Shortcuts> <Shortcut id="3027" /> <Shortcut id="3415" /> </Shortcuts> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="3030" reactomeId="113529" position="634 551" bounds="616 536 36 31" textPosition="622 540" /> <org.gk.render.RenderableComplex id="3031" reactomeId="9912540" position="944 468" bounds="901 457 87 23" textPosition="904 458" bgColor="204 255 255" hideComponents="true"> <Properties> <displayName>BCKDK dimer</displayName> </Properties> </org.gk.render.RenderableComplex> <org.gk.render.RenderableChemical id="3064" reactomeId="113581" position="1027 499" bounds="1007 484 41 31" textPosition="1012 488"> <Properties> <displayName>ADP</displayName> </Properties> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="3069" reactomeId="113581" position="536 1138" bounds="516 1123 41 31" textPosition="521 1127" /> <org.gk.render.RenderableChemical id="3071" reactomeId="113529" position="519 1178" bounds="501 1163 36 31" textPosition="507 1167" /> <org.gk.render.RenderableChemical id="3073" reactomeId="113529" position="590 918" bounds="572 903 36 31" textPosition="578 907"> <Properties> <displayName>H+</displayName> </Properties> <Shortcuts> <Shortcut id="3073" /> <Shortcut id="3422" /> </Shortcuts> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="3079" reactomeId="113529" position="1072 533" bounds="1054 518 36 31" textPosition="1060 522" /> <org.gk.render.RenderableChemical id="3085" reactomeId="113581" position="774 1192" bounds="754 1177 41 31" textPosition="759 1181" /> <org.gk.render.RenderableChemical id="3087" reactomeId="113529" position="739 1210" bounds="721 1195 36 31" textPosition="727 1199" /> <org.gk.render.RenderableChemical id="3097" reactomeId="113529" position="825 901" bounds="807 886 36 31" textPosition="813 890" /> <org.gk.render.RenderableChemical id="3107" reactomeId="113529" position="1052 906" bounds="1034 891 36 31" textPosition="1040 895" /> <org.gk.render.RenderableChemical id="3117" reactomeId="113529" position="1075 1246" bounds="1057 1231 36 31" textPosition="1063 1235" /> <org.gk.render.RenderableChemical id="3126" reactomeId="113521" position="1154 1510" bounds="1133 1495 43 31" textPosition="1138 1499" /> <org.gk.render.RenderableChemical id="3405" reactomeId="29896" position="963 1560" bounds="934 1545 58 31" textPosition="941 1549" /> <org.gk.render.RenderableChemical id="3407" reactomeId="113521" position="1060 645" bounds="1039 630 43 31" textPosition="1044 634" /> <org.gk.render.RenderableChemical id="3409" reactomeId="113548" position="871 647" bounds="858 632 27 31" textPosition="862 636"> <Properties> <displayName>Pi</displayName> </Properties> </org.gk.render.RenderableChemical> <org.gk.render.RenderableChemical id="3411" reactomeId="201023" position="1253 857" bounds="1233 842 40 31" textPosition="1237 846" /> <org.gk.render.RenderableChemical id="3412" reactomeId="9971124" position="1219 687" bounds="1187 672 64 31" textPosition="1191 676" /> <org.gk.render.RenderableProtein id="3413" reactomeId="9971122" position="1316 778" bounds="1294 766 44 25" textPosition="1295 767" /> <org.gk.render.RenderableChemical id="3415" reactomeId="29374" position="1107 736" bounds="1076 721 62 31" textPosition="1082 725" /> <org.gk.render.RenderableChemical id="3418" reactomeId="159549" position="948 843" bounds="932 828 32 31" textPosition="936 832" /> <org.gk.render.RenderableChemical id="3419" reactomeId="9971623" position="906 730" bounds="878 715 56 31" textPosition="882 719" /> <org.gk.render.RenderableProtein id="3420" reactomeId="159545" position="1001 804" bounds="978 792 46 25" textPosition="978 793" /> <org.gk.render.RenderableChemical id="3422" reactomeId="113529" position="838 738" bounds="820 723 36 31" textPosition="826 727" /> </Nodes> <Edges> <org.gk.render.RenderableReaction id="2363" reactomeId="70723" points="190 153, 190 177, 190 201" position="190 177" lineWidth="1.0"> <Inputs> <Input id="140" points="443 121" /> <Input id="146" points="189 136" /> </Inputs> <Outputs> <Output id="141" points="301 232" /> <Output id="144" points="190 234" /> </Outputs> <Catalysts> <Catalyst id="142" points="214 177" /> </Catalysts> <Properties> <displayName>leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1]</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2364" reactomeId="508189" points="334 197, 334 178, 334 155" position="334 178" lineWidth="1.0"> <Inputs> <Input id="141" points="335 232" /> <Input id="144" points="218 242" /> </Inputs> <Outputs> <Output id="140" points="443 126" /> <Output id="146" points="214 122" /> </Outputs> <Catalysts> <Catalyst id="142" points="306 178" /> </Catalysts> <Properties> <displayName>a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT1]</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2365" reactomeId="70724" points="744 298, 745 317, 744 333" position="745 317" lineWidth="1.0"> <Inputs> <Input id="150" points="744 266" /> <Input id="602" points="620 248" /> </Inputs> <Outputs> <Output id="151" points="744 382" /> <Output id="156" points="625 371" /> </Outputs> <Catalysts> <Catalyst id="152" points="714 316" /> </Catalysts> <Properties> <displayName>leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2366" reactomeId="508179" points="596 335, 597 318, 596 301" position="597 318" lineWidth="1.0"> <Inputs> <Input id="151" points="717 382" /> <Input id="156" points="596 361" /> </Inputs> <Outputs> <Output id="150" points="711 266" /> <Output id="602" points="595 259" /> </Outputs> <Catalysts> <Catalyst id="152" points="622 317" /> </Catalysts> <Properties> <displayName>a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2]</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2370" reactomeId="70745" points="624 933, 623 961, 623 992" position="623 961" lineWidth="1.0"> <Inputs> <Input id="172" points="619 900" /> <Input id="202" points="664 912" /> <Input id="3073" points="611 925" /> </Inputs> <Outputs> <Output id="173" points="648 1032" /> <Output id="200" points="668 1002" /> </Outputs> <Catalysts> <Catalyst id="175" points="587 961" /> </Catalysts> <Properties> <displayName>isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2371" reactomeId="70773" points="582 1127, 582 1150, 583 1175" position="582 1150" lineWidth="1.0"> <Inputs> <Input id="173" points="642 1069" /> <Input id="177" points="728 1077" /> <Input id="898" points="574 1093" /> </Inputs> <Outputs> <Output id="180" points="643 1193" /> <Output id="178" points="644 1233" /> <Output id="3069" points="555 1157" /> <Output id="3071" points="540 1177" /> </Outputs> <Catalysts> <Catalyst id="182" points="608 1148" /> </Catalysts> <Properties> <displayName>beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2376" reactomeId="508308" points="743 1175, 742 1153, 742 1120" position="742 1153" lineWidth="1.0"> <Inputs> <Input id="180" points="676 1194" /> <Input id="178" points="676 1233" /> <Input id="3085" points="751 1180" /> <Input id="3087" points="740 1192" /> </Inputs> <Outputs> <Output id="173" points="676 1069" /> <Output id="898" points="604 1081" /> <Output id="177" points="748 1090" /> </Outputs> <Catalysts> <Catalyst id="182" points="713 1150" /> </Catalysts> <Properties> <displayName>beta-methylglutaconyl-CoA + ADP + orthophosphate <=> beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA)</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2382" reactomeId="70785" points="717 1281, 717 1314, 717 1345" position="717 1314" lineWidth="1.0"> <Inputs> <Input id="178" points="689 1270" /> <Input id="179" points="730 1265" /> </Inputs> <Outputs> <Output id="193" /> </Outputs> <Catalysts> <Catalyst id="194" points="658 1313" /> </Catalysts> <Properties> <displayName>beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2386" reactomeId="70800" points="776 937, 776 963, 776 990" position="776 963" lineWidth="1.0"> <Inputs> <Input id="196" points="763 891" /> <Input id="202" points="710 910" /> <Input id="3097" points="804 914" /> </Inputs> <Outputs> <Output id="197" points="813 1006" /> <Output id="200" points="731 1001" /> </Outputs> <Catalysts> <Catalyst id="198" points="750 961" /> </Catalysts> <Properties> <displayName>alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2387" reactomeId="70830" points="906 1060, 906 1090, 906 1126" position="906 1090" lineWidth="1.0"> <Inputs> <Input id="197" points="871 1043" /> <Input id="206" points="980 1044" /> </Inputs> <Outputs> <Output id="207" /> </Outputs> <Catalysts> <Catalyst id="208" points="941 1090" /> </Catalysts> <Properties> <displayName>tiglyl-CoA + H2O <=> alpha-methyl-beta-hydroxybutyryl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2391" reactomeId="70837" points="1008 1216, 1007 1235, 1008 1253" position="1007 1235" lineWidth="1.0"> <Inputs> <Input id="207" points="928 1163" /> <Input id="215" points="939 1198" /> </Inputs> <Outputs> <Output id="210" points="943 1317" /> <Output id="217" points="1004 1291" /> <Output id="213" points="958 1264" /> </Outputs> <Catalysts> <Catalyst id="211" points="988 1234" /> </Catalysts> <Properties> <displayName>alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + NADH + H+</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2392" reactomeId="508369" points="831 1261, 831 1235, 831 1204" position="831 1235" lineWidth="1.0"> <Inputs> <Input id="210" points="908 1317" /> <Input id="213" points="916 1266" /> <Input id="217" points="974 1301" /> </Inputs> <Outputs> <Output id="207" points="887 1163" /> <Output id="215" points="880 1196" /> </Outputs> <Catalysts> <Catalyst id="211" points="856 1234" /> </Catalysts> <Properties> <displayName>alpha-methylacetoacetyl-CoA + NADH + H+ <=> alpha-methyl-beta-hydroxybutyryl-CoA + NAD+</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2395" reactomeId="70844" points="916 1374, 916 1393, 916 1414" position="916 1393" lineWidth="1.0"> <Inputs> <Input id="210" points="923 1354" /> <Input id="226" points="854 1350" /> </Inputs> <Outputs> <Output id="222" points="942 1428" /> <Output id="223" points="891 1429" /> </Outputs> <Catalysts> <Catalyst id="224" points="891 1392" /> </Catalysts> <Properties> <displayName>alpha-methylacetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2398" reactomeId="70859" points="970 912, 969 932, 969 952" position="969 932" lineWidth="1.0"> <Inputs> <Input id="229" points="944 898" /> <Input id="3107" points="1031 907" /> <Input id="234" points="985 904" /> </Inputs> <Outputs> <Output id="230" points="1065 989" /> <Output id="236" points="939 968" /> </Outputs> <Catalysts> <Catalyst id="231" points="927 934" /> </Catalysts> <Properties> <displayName>isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2399" reactomeId="70870" points="1106 1053, 1106 1091, 1106 1123" position="1106 1091" lineWidth="1.0"> <Inputs> <Input id="230" points="1104 1026" /> <Input id="206" points="1029 1042" /> </Inputs> <Outputs> <Output id="239" /> </Outputs> <Catalysts> <Catalyst id="2789" points="1133 1090" /> </Catalysts> <Properties> <displayName>ECHS1 hydrates methacrylyl-CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2401" reactomeId="70881" points="1157 1179, 1157 1205, 1157 1228" position="1157 1205" lineWidth="1.0"> <Inputs> <Input id="239" points="1127 1160" /> <Input id="242" points="1177 1163" /> </Inputs> <Outputs> <Output id="885" points="1139 1246" /> <Output id="246" points="1186 1242" /> <Output id="3117" points="1096 1242" /> </Outputs> <Catalysts> <Catalyst id="244" points="1188 1204" /> </Catalysts> <Properties> <displayName>beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2405" reactomeId="70885" points="1223 1350, 1222 1375, 1223 1396" position="1222 1375" lineWidth="1.0"> <Inputs> <Input id="885" points="1143 1283" /> <Input id="254" points="1166 1331" /> </Inputs> <Outputs> <Output id="248" points="1159 1453" /> <Output id="256" points="1225 1447" /> <Output id="252" points="1174 1402" /> </Outputs> <Catalysts> <Catalyst id="249" points="1201 1374" /> </Catalysts> <Properties> <displayName>beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2410" reactomeId="508473" points="1065 1394, 1065 1376, 1065 1348" position="1065 1376" lineWidth="1.0"> <Inputs> <Input id="248" points="1127 1453" /> <Input id="252" points="1132 1402" /> <Input id="256" points="1197 1453" /> </Inputs> <Outputs> <Output id="885" points="1114 1283" /> <Output id="254" points="1107 1332" /> </Outputs> <Catalysts> <Catalyst id="249" points="1087 1375" /> </Catalysts> <Properties> <displayName>methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2413" reactomeId="70893" points="1067 1485, 1039 1485, 1000 1486" position="1039 1485" lineWidth="1.0"> <Inputs> <Input id="3126" points="1130 1503" /> <Input id="248" points="1098 1478" /> <Input id="265" points="1125 1531" /> <Input id="263" points="1061 1457" /> </Inputs> <Outputs> <Output id="222" points="980 1465" /> <Output id="268" points="940 1490" /> <Output id="272" points="934 1524" /> <Output id="3405" points="970 1542" /> </Outputs> <Catalysts> <Catalyst id="261" points="1039 1518" /> </Catalysts> <Properties> <displayName>methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2775" reactomeId="5693148" points="909 549, 946 549, 981 549" position="946 549" lineWidth="1.0"> <Inputs> <Input id="2776" stoichiometry="2" points="890 530" /> <Input id="166" points="886 560" /> </Inputs> <Outputs> <Output id="2778" points="1012 563" /> <Output id="3064" stoichiometry="2" points="1012 518" /> <Output id="3079" points="1051 536" /> </Outputs> <Catalysts> <Catalyst id="3031" points="944 483" /> </Catalysts> <Properties> <displayName>BCKDK phosphorylates BCKDH</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2780" reactomeId="5693153" points="990 605, 953 605, 911 604" position="953 605" lineWidth="1.0"> <Inputs> <Input id="3407" stoichiometry="2" points="1036 632" /> <Input id="2778" points="1016 592" /> </Inputs> <Outputs> <Output id="166" points="883 589" /> <Output id="3409" stoichiometry="2" points="885 629" /> </Outputs> <Catalysts> <Catalyst id="2782" points="954 647" /> </Catalysts> <Properties> <displayName>PPM1K dephosphorylates p-BCKDH</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="2785" reactomeId="9672770" points="531 125, 744 174, 744 231" position="744 174" lineWidth="1.0"> <Inputs> <Input id="140" /> </Inputs> <Outputs> <Output id="150" /> </Outputs> <Catalysts> <Catalyst id="2786" points="777 173" /> </Catalysts> <Properties> <displayName>SLC25A44 transports Leu, Ile and Val from cytosol to mitochondrial matrix</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.EntitySetAndMemberLink id="2997" points="756 854, 749 816" position="752 835" lineWidth="0.5" lineColor="0 0 0"> <Inputs> <Input id="196" /> </Inputs> <Outputs> <Output id="2996" /> </Outputs> </org.gk.render.EntitySetAndMemberLink> <org.gk.render.EntitySetAndMemberLink id="2998" points="877 867, 769 816" position="823 841" lineWidth="0.5" lineColor="0 0 0"> <Inputs> <Input id="229" /> </Inputs> <Outputs> <Output id="2996" /> </Outputs> </org.gk.render.EntitySetAndMemberLink> <org.gk.render.EntitySetAndMemberLink id="2999" points="643 863, 729 816" position="686 839" lineWidth="0.5" lineColor="0 0 0"> <Inputs> <Input id="172" /> </Inputs> <Outputs> <Output id="2996" /> </Outputs> </org.gk.render.EntitySetAndMemberLink> <org.gk.render.RenderableReaction id="3016" reactomeId="9859148" points="743 472, 744 495, 743 517" position="744 495" lineWidth="1.0"> <Inputs> <Input id="3017" points="662 451" /> <Input id="151" points="743 417" /> <Input id="3022" points="812 428" /> </Inputs> <Outputs> <Output id="3018" points="743 560" /> <Output id="3020" points="712 529" /> </Outputs> <Catalysts> <Catalyst id="166" points="849 560" /> </Catalysts> <Properties> <displayName>BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="3024" reactomeId="9859163" points="742 642, 743 689, 743 713" position="743 689" lineWidth="1.0"> <Inputs> <Input id="3018" points="743 609" /> <Input id="3027" points="710 634" /> </Inputs> <Outputs> <Output id="3025" points="676 728" /> <Output id="2996" points="747 781" /> </Outputs> <Catalysts> <Catalyst id="166" points="859 589, 791 688" /> </Catalysts> <Properties> <displayName>DBT transfers BCAA to CoA</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="3028" reactomeId="9859172" points="583 618, 583 589, 584 561" position="583 589" lineWidth="1.0"> <Inputs> <Input id="3025" points="620 728" /> <Input id="3011" points="560 640" /> </Inputs> <Outputs> <Output id="3017" points="621 451" /> <Output id="3030" points="613 554" /> <Output id="3007" points="563 542" /> </Outputs> <Catalysts> <Catalyst id="166" points="850 589, 705 685, 645 685" /> </Catalysts> <Properties> <displayName>DLD dimer dehydrogenates dihydrolipoyl</displayName> </Properties> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="3410" reactomeId="9971123" points="1218 809, 1217 779, 1216 747" position="1217 779" lineWidth="1.0"> <Inputs> <Input id="229" points="944 877" /> <Input id="3411" points="1241 839" /> </Inputs> <Outputs> <Output id="3412" points="1218 706" /> <Output id="3415" points="1141 739" /> </Outputs> <Catalysts> <Catalyst id="3413" points="1291 778" /> </Catalysts> </org.gk.render.RenderableReaction> <org.gk.render.RenderableReaction id="3417" reactomeId="9971621" points="909 826, 908 804, 907 779" position="908 804" lineWidth="1.0"> <Inputs> <Input id="229" points="909 867" /> <Input id="3418" points="929 836" /> </Inputs> <Outputs> <Output id="3419" points="906 749" /> <Output id="3422" points="859 748" /> <Output id="3415" points="1073 742" /> </Outputs> <Catalysts> <Catalyst id="3420" points="975 804" /> </Catalysts> </org.gk.render.RenderableReaction> </Edges> <Pathways /> </Process> |
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No pathways have been reviewed or authored by Diagram of Branched-chain amino acid catabolism, Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD, Loss-of-function mutations in DBT cause MSUD2, Loss-of-function mutations in DLD cause MSUD3/DLDD, H139Hfs13* PPM1K causes a mild variant of MSUD, Branched-chain ketoacid dehydrogenase kinase deficiency, Isovaleric acidemia, 3-Methylcrotonyl-CoA carboxylase deficiency, 3-methylglutaconic aciduria, Beta-ketothiolase deficiency, Mitochondrial short-chain enoyl-CoA hydratase deficiency 1, and 3-hydroxyisobutyryl-CoA hydrolase deficiency (6788658)
