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Details on Person UniProt:Q86X52 CHSY1

Class:IdReferenceGeneProduct:223471
_chainChangeLogchain:1-802 added on Sat February 7 2015
_displayNameUniProt:Q86X52 CHSY1
_timestamp2025-02-21 19:26:18
chainchain:1-802
checksum5B4C02670332FA0E
commentFUNCTION Has both beta-1,3-glucuronic acid and beta-1,4-N-acetylgalactosamine transferase activity. Transfers glucuronic acid (GlcUA) from UDP-GlcUA and N-acetylgalactosamine (GalNAc) from UDP-GalNAc to the non-reducing end of the elongating chondroitin polymer. Involved in the negative control of osteogenesis likely through the modulation of NOTCH signaling.CATALYTIC ACTIVITY 3-O-(beta-D-GlcA-(1->3)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP-N-acetyl-alpha-D-galactosamine = 3-O-(beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP + H(+)CATALYTIC ACTIVITY 3-O-{beta-D-GlcA-(1->3)-[beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP-N-acetyl-alpha-D-galactosamine = 3-O-{[beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n+1)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP + H(+)CATALYTIC ACTIVITY 3-O-(beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP-alpha-D-glucuronate = 3-O-(beta-D-GlcA-(1->3)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP + H(+)CATALYTIC ACTIVITY 3-O-{[beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP-alpha-D-glucuronate = 3-O-{beta-D-GlcA-(1->3)-[beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP + H(+)COFACTOR Divalent metal cations. Highest activities are measured with Co(2+), Mn(2+) and Cd(2+).SUBCELLULAR LOCATION Ubiquitous, with the highest levels in placenta. Detected at low levels in brain, heart, skeletal muscle, colon, thymus, spleen, kidney, liver, adrenal gland, mammary gland, stomach, small intestine, lung and peripheral blood leukocytes.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the chondroitin N-acetylgalactosaminyltransferase family.SEQUENCE CAUTION Extended N-terminus.ONLINE INFORMATION Chondroitin sulfate synthase 1
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: fullName evidence="8"Chondroitin sulfate synthase 1 ecNumber evidence="4"2.4.1.175 ecNumber evidence="4"2.4.1.226 alternativeName: Chondroitin glucuronyltransferase 1 alternativeName: Chondroitin synthase 1 shortName: ChSy-1 alternativeName: Glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase 1 alternativeName: N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase 1 alternativeName: N-acetylgalactosaminyltransferase 1
geneNameCHSY1
CHSY
CSS1
KIAA0990
UNQ756/PRO1487
identifierQ86X52
isSequenceChangedFALSE
keywordDisease variant
Glycoprotein
Golgi apparatus
Membrane
Metal-binding
Proteomics identification
Reference proteome
Secreted
Signal-anchor
Transferase
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameCHSY1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9002859] ENSEMBL:ENSG00000131873 CHSY1 [Homo sapiens]
secondaryIdentifierCHSS1_HUMAN
Q6UX38
Q7LFU5
Q9Y2J5
sequenceLength802
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:1971431] CHSY1 [Golgi membrane] [Homo sapiens]
[EntityWithAccessionedSequence:3636823] CHSY1 G19_L28del [Golgi membrane] [Homo sapiens]
[EntityWithAccessionedSequence:3636838] CHSY1 Q69* [Golgi membrane] [Homo sapiens]
[EntityWithAccessionedSequence:3636862] CHSY1 P539R [Golgi membrane] [Homo sapiens]
[EntityWithAccessionedSequence:3636864] CHSY1 E33Sfs*2 [Golgi membrane] [Homo sapiens]
(referenceSequence)[NonsenseMutation:3636791] Nonsense mutation at L-glutamine 69
[FragmentDeletionModification:3636808] Deletion of residues 19 to 28
[ReplacedResidue:3636810] L-proline 539 replaced with L-arginine
[FragmentReplacedModification:3636866] Replacement of residues 33 to 33 by S
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No pathways have been reviewed or authored by UniProt:Q86X52 CHSY1 (223471)
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