HS3ST3A1

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Description

The HS3ST3A1 (heparan sulfate-glucosamine 3-sulfotransferase 3A1) is a protein-coding gene located on chromosome 17.

Heparan sulfate glucosamine 3-O-sulfotransferase 3A1 is an enzyme encoded by the HS3ST3A1 gene in humans. It is part of the heparan sulfate biosynthetic enzyme family and plays a key role in generating diverse heparan sulfate structures, which are involved in various biological activities. This enzyme is a type II integral membrane protein with heparan sulfate glucosaminyl 3-O-sulfotransferase activity. Its sulfotransferase domain is highly similar to the same domain of heparan sulfate D-glucosaminyl 3-O-sulfotransferase 3A1, and both enzymes sulfate the same disaccharide. HS3ST3A1 is expressed widely, with the highest levels found in the liver and placenta.

Heparan sulfate glucosamine 3-O-sulfotransferase 3A1 (HS3ST3A1) utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) to transfer a sulfo group to an N-unsubstituted glucosamine linked to a 2-O-sulfo iduronic acid unit on heparan sulfate. It catalyzes the O-sulfation of glucosamine in IdoUA2S-GlcNS and IdoUA2S-GlcNH2. This specific O-sulfation generates an enzyme-modified heparan sulfate that acts as a binding receptor for Herpes simplex virus-1 (HSV-1), facilitating its entry. Unlike HS3ST1/3-OST-1, HS3ST3A1 does not convert non-anticoagulant heparan sulfate to anticoagulant heparan sulfate.

HS3ST3A1 is also known as 3-OST-3A, 3OST3A1.

Associated Diseases

• beta-thalassemia-X-linked thrombocytopenia syndrome
• dilated cardiomyopathy 1I
• dehydrated hereditary stomatocytosis
• pentosuria
• bleeding disorder, platelet-type, 24
• platelet-type bleeding disorder 15
• thrombocytopenia 7
• sitosterolemia
• DIAPH1-related sensorineural hearing loss-thrombocytopenia syndrome
• thrombocytopenia 4
• thrombocytopenia 2
• Fanconi anemia complementation group V
• cystic fibrosis