Aliases for PAPSS1 Gene
External Ids for PAPSS1 Gene
Previous GeneCards Identifiers for PAPSS1 Gene
Three-prime-phosphoadenosine 5-prime-phosphosulfate (PAPS) is the sulfate donor cosubstrate for all sulfotransferase (SULT) enzymes (Xu et al., 2000 [PubMed 10679223]). SULTs catalyze the sulfate conjugation of many endogenous and exogenous compounds, including drugs and other xenobiotics. In humans, PAPS is synthesized from adenosine 5-prime triphosphate (ATP) and inorganic sulfate by 2 isoforms, PAPSS1 and PAPSS2 (MIM 603005).[supplied by OMIM, Mar 2008]
GeneCards Summary for PAPSS1 Gene
PAPSS1 (3'-Phosphoadenosine 5'-Phosphosulfate Synthase 1) is a Protein Coding gene. Diseases associated with PAPSS1 include Spondyloepimetaphyseal Dysplasia and Achondrogenesis, Type Ib. Among its related pathways are Viral mRNA Translation and Sulfation Biotransformation Reaction. Gene Ontology (GO) annotations related to this gene include nucleotidyltransferase activity and sulfate adenylyltransferase (ATP) activity. An important paralog of this gene is PAPSS2.
UniProtKB/Swiss-Prot Summary for PAPSS1 Gene
Bifunctional enzyme with both ATP sulfurylase and APS kinase activity, which mediates two steps in the sulfate activation pathway. The first step is the transfer of a sulfate group to ATP to yield adenosine 5'-phosphosulfate (APS), and the second step is the transfer of a phosphate group from ATP to APS yielding 3'-phosphoadenylylsulfate (PAPS: activated sulfate donor used by sulfotransferase). In mammals, PAPS is the sole source of sulfate; APS appears to be only an intermediate in the sulfate-activation pathway (PubMed:9576487, PubMed:9668121, PubMed:9648242, PubMed:14747722). Required for normal biosynthesis of sulfated L-selectin ligands in endothelial cells (PubMed:9576487).