Chst11

Chondroitin 4-sulfotransferases, such as CHST11, catalyze the transfer of sulfate from 3-prime-phosphoadenosine 5-prime-phosphosulfate to position 4 of N-acetylgalactosamine residues in chondroitin (Yamauchi et al., 2000 [PubMed 10722746]).[supplied by OMIM

Full Name

carbohydrate (chondroitin 4) sulfotransferase 11

Function

Catalyzes the transfer of sulfate to position 4 of the N-acetylgalactosamine (GalNAc) residue of chondroitin. Chondroitin sulfate constitutes the predominant proteoglycan present in cartilage and is distributed on the surfaces of many cells and extracellular matrices. Can also sulfate Gal residues in desulfated dermatan sulfate. Preferentially sulfates in GlcA->GalNAc unit than in IdoA->GalNAc unit. Does not form 4, 6-di-O-sulfated GalNAc when chondroitin sulfate C is used as an acceptor.

Biological Process

Carbohydrate biosynthetic process Source: InterPro
Chondrocyte development Source: Ensembl
Chondroitin sulfate biosynthetic process Source: UniProtKB
Developmental growth Source: Ensembl
Embryonic digit morphogenesis Source: Ensembl
Embryonic viscerocranium morphogenesis Source: Ensembl
In utero embryonic development Source: Ensembl
Negative regulation of apoptotic process Source: Ensembl
Negative regulation of transforming growth factor beta receptor signaling pathway Source: Ensembl
Polysaccharide localization Source: Ensembl
Post-anal tail morphogenesis Source: Ensembl
Post-embryonic development Source: Ensembl
Proteoglycan biosynthetic process Source: GO_Central
Regulation of cell population proliferation Source: Ensembl
Respiratory gaseous exchange by respiratory system Source: Ensembl

Cellular Location

Golgi apparatus membrane

Involvement in disease

A chromosomal aberration involving CHST11 is found in B-cell chronic lymphocytic leukemias. Translocation t(12;14)(q23;q32) with IgH.
Osteochondrodysplasia, brachydactyly, and overlapping malformed digits (OCBMD):
An autosomal recessive disorder characterized by bilateral symmetric skeletal defects that primarily affect the limbs. Affected individuals have mild short stature due to shortening of the lower leg bones, as well as hand and foot malformations, predominantly brachydactyly and overlapping digits. Other skeletal defects include scoliosis, dislocated patellae and fibulae, and pectus excavatum.

Topology

Cytoplasmic: 1-16
Helical: 17-37
Lumenal: 38-352

PTM

N-glycosylated; required for activity and stability.