PAPSS2


Description

The PAPSS2 (3'-phosphoadenosine 5'-phosphosulfate synthase 2) is a protein-coding gene located on chromosome 10.

The PAPSS2 gene encodes bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetase 2, an enzyme involved in the production of 3'-phosphoadenosine 5'-phosphosulfate (PAPS). PAPS is a crucial molecule in sulfation, a common modification of various compounds, including lipids, proteins, carbohydrates, xenobiotics, and drugs. Defects in the PAPSS2 gene are linked to Pakistani type spondyloepimetaphyseal dysplasia. The gene has two alternatively spliced transcript variants, producing different isoforms.

PAPSS2 is a bifunctional enzyme that catalyzes two steps in the sulfate activation pathway: (1) transfer of a sulfate group from ATP to adenosine 5'-phosphosulfate (APS) and (2) transfer of a phosphate group from ATP to APS yielding 3'-phosphoadenylylsulfate (PAPS). PAPS is the primary sulfate donor for sulfotransferases, which modify a variety of endogenous and exogenous compounds. PAPSS2 indirectly contributes to skeletogenesis during postnatal growth.

PAPSS2 is also known as ATPSK2, BCYM4, SK2.

Associated Diseases


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