CHST14
Description
The CHST14 (carbohydrate sulfotransferase 14) is a protein-coding gene located on chromosome 15.
CHST14, a protein-coding gene, encodes for the enzyme carbohydrate sulfotransferase 14 (CHST14)/dermatan 4-O-sulfotransferase (D4ST1). Located on chromosome 15, CHST14 spans 3,611 bases, encodes 376 amino acids, and has a molecular mass of 42997 Da. This gene plays a critical role in fetal development of connective tissues across various organ systems, including regulation of proliferation and neurogenesis of neural precursor cells. However, it has also been linked to inhibiting peripheral nerve regeneration in adults. CHST14's primary function involves the transfer of an active sulfate to position 4 of N-acetyl-D-galactosamine residues within dermatan sulfate. This process stabilizes dermatan sulfate, a glycosaminoglycan essential for extracellular matrix formation, found abundantly in skin, tendons, cartilage, and the aortic wall. Mutations in CHST14 lead to dermatan sulfate deficiency, disrupting glycosaminoglycan constituents in fibroblasts and impairing collagen fibril linkage within collagen bundles.
CHST14 catalyzes the transfer of a sulfate group to position 4 of N-acetylgalactosamine (GalNAc) in dermatan sulfate. This modification is crucial for the formation of 4-O-sulfated IdoA blocks, which are essential components of dermatan sulfate. The enzyme preferentially adds sulfate to GalNAc residues within -IdoUA-GalNAc-IdoUA- sequences, suggesting a role in the regulation of dermatan sulfate structure. Notably, CHST14 displays a preference for partially desulfated dermatan sulfate, indicating a potential role in the fine-tuning of sulfation patterns. Moreover, CHST14's activity may be closely linked to the epimerization of GlcUA to IdoUA, suggesting a coordinated process in dermatan sulfate biosynthesis. Importantly, CHST14 is implicated in the development of the cerebellar neural network during postnatal brain development, highlighting its role in neuronal maturation and function.
CHST14 is also known as ATCS, D4ST1, EDSMC1, HNK1ST.
