Last Update:2021-12-17

CHSY1 encodes chondroitin sulfate synthase 1, which is involved in the biosynthesis of chondroitin sulfate. CHSY1 has two glycosyltransferase domains, β1,3GT domain and β1,4GT domain, and forms a complex with CHSY3, CHPF, and CHPF2 to exert GalNAc-transferase and GlcA-transferase activities, which are thought to be responsible for the elongation of chondroitin chains. Various combinations of CHSY, CHSY3, CHPF, and CHPF2 have been reported to produce different lengths of chondroitin chains. CHSY1 is the causative gene of temtamy preaxial brachydactyly syndrome.

Keyword: chondroitin ,  chondroitin sulfate ,  proteoglycan

N-Acetylgalactosaminyltransferase Glucuronyltransferase

GGDB Symbol CHSY1    Alias
Designationchondroitin sulfate synthase 1, glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase, N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase 1
OrganismHomo sapiens
GeneID 22856
HGNC CHSY1 (17198)
mRNA NM_014918
Protein NP_055733
CAZy GT31   
GlyCosmos Glycogenes CHSY1
OMIM 608183
Disease name
Human Protein Atlas ENSG00000131873
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Orthologous Gene

Acceptor Substrates (Reference)

Acceptor Substrates (KEM-C)


RNA Expression from Human Protein Atlas
This RNA expression data uses "HPA data" from the Human Protein Atlas. Tissue RNA Expression

data available from

Expression (Reference)

Gene Ontology

This page does not indicate all of the enzymatic reaction, and expression of "CHSY1".