PAPSS1

Description

The PAPSS1 (3'-phosphoadenosine 5'-phosphosulfate synthase 1) is a protein-coding gene located on chromosome 4.

PAPSS1 is a human gene that encodes bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetase 1, an enzyme that produces 3'-phosphoadenosine 5'-phosphosulfate (PAPS), the sulfate donor cosubstrate for all sulfotransferase (SULT) enzymes. SULTs catalyze the sulfate conjugation of many endogenous and exogenous compounds, including drugs and other xenobiotics.

PAPSS1 is a bifunctional enzyme that catalyzes two steps in the sulfate activation pathway. First, it transfers a sulfate group from ATP to yield adenosine 5'-phosphosulfate (APS). Second, it transfers a phosphate group from ATP to APS, yielding 3'-phosphoadenylylsulfate (PAPS), the activated sulfate donor used by sulfotransferases. In mammals, PAPS is the sole source of sulfate, and APS is an intermediate in the sulfate activation pathway. PAPSS1 is required for normal biosynthesis of sulfated L-selectin ligands in endothelial cells. {ECO:0000269|PubMed:14747722, ECO:0000269|PubMed:9576487, ECO:0000269|PubMed:9648242, ECO:0000269|PubMed:9668121}

PAPSS1 is also known as ATPSK1, PAPSS, SK1.

Associated Diseases

WES Whole Exome Sequencing