CHSY1 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. Belongs to the chondroitin N-acetylgalactosaminyltransferase family. 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. Note: This description may include information from UniProtKB.
Protein type: EC; EC; Glycan Metabolism - chondroitin sulfate biosynthesis; Membrane protein, integral; Transferase
Chromosomal Location of human Ortholog: 15q26.3
Cellular Component:  extracellular region; Golgi cisterna membrane; Golgi membrane; integral component of membrane
Molecular Function:  glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase activity; metal ion binding; N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase activity
Biological Process:  bone morphogenesis; chondrocyte development; chondroitin sulfate biosynthetic process; negative regulation of ossification; positive regulation of smoothened signaling pathway; proximal/distal pattern formation; response to nutrient levels; sulfation
Disease: Temtamy Preaxial Brachydactyly Syndrome
Reference #:  Q86X52 (UniProtKB)
Alt. Names/Synonyms: carbohydrate synthase 1; Chondroitin glucuronyltransferase 1; chondroitin glucuronyltransferase II; Chondroitin sulfate synthase 1; chondroitin synthase; Chondroitin synthase 1; CHSS1; CHSY; ChSy-1; CHSY1; CSS1; DKFZp434M032; FLJ58581; Glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase 1; KIAA0990; N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase 1; N-acetylgalactosaminyltransferase 1; N-acetylgalactosaminyltransferase II; TPBS
Gene Symbols: CHSY1
Molecular weight: 91,784 Da
Basal Isoelectric point: 9.31  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene