CHST2

CHST2

The protein structure of CHST2.

CHST2 (Carbohydrate Sulfotransferase 2) is an enzyme that plays a crucial role in the sulfation of carbohydrates. It is encoded by the CHST2 gene, which is located on chromosome 20 in humans. CHST2 belongs to the sulfotransferase family and is primarily expressed in various tissues, including the brain, liver, and kidney.

Structure

The CHST2 protein consists of 389 amino acids and has a molecular weight of approximately 44 kDa. It contains a conserved sulfotransferase domain, which is responsible for catalyzing the transfer of sulfate groups from a donor molecule to specific carbohydrate substrates. The protein structure of CHST2 has been determined through X-ray crystallography, revealing its active site and binding regions.

Function

CHST2 is involved in the sulfation of carbohydrates, specifically the addition of sulfate groups to the 4-O position of N-acetylgalactosamine (GalNAc) residues. This sulfation process is crucial for the biosynthesis of sulfated glycosaminoglycans (GAGs), which are important components of the extracellular matrix and play essential roles in cell signaling, tissue development, and homeostasis.

The sulfation of GalNAc residues by CHST2 is a key step in the biosynthesis of chondroitin sulfate, a major GAG found in cartilage, tendons, and other connective tissues. Chondroitin sulfate provides structural support and elasticity to these tissues, contributing to their proper function.

Clinical Significance

Mutations in the CHST2 gene have been associated with various diseases and conditions. For example, deficiencies in CHST2 activity can lead to abnormal sulfation of chondroitin sulfate, resulting in skeletal abnormalities and joint disorders such as spondyloepiphyseal dysplasia and osteoarthritis.

Furthermore, altered expression levels of CHST2 have been observed in certain cancers, including breast, lung, and colorectal cancer. CHST2 has been implicated in tumor progression and metastasis, suggesting its potential as a therapeutic target for cancer treatment.

See Also

• Carbohydrate sulfotransferase
• Glycosaminoglycans
• Chondroitin sulfate
• Spondyloepiphyseal dysplasia
• Osteoarthritis
• Cancer