bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2; PAPS synthase 2; PAPSS 2; sulfurylase kinase 2; SK 2; SK2; includes: sulfate adenylyltransferase; adenylyl-sulfate kinase; 3'-phosphoadenosine-5'-phosphosulfate synthase; APS kinase; adenosine-5'-phosphosulfate 3'-phosphotransferase; adenylylsulfate 3'-phosphotransferase (PAPSS2, ATPSK2)

Function: - bifunctional enzyme with both ATP sulfurylase & 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) - 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 - may have a important role in skeletogenesis during postnatal growth - sulfur metabolism; sulfate assimilation ATP + sulfate = diphosphate + adenylyl sulfate ATP + adenylyl sulfate = ADP + 3'-phosphoadenylyl sulfate Structure: - in the N-terminal section; belongs to the APS kinase family - in the C-terminal section; belongs to the sulfate adenylyltransferase family Alternative splicing: named isoforms=2 Expression: - expressed in cartilage & adrenal gland Pathology: - defects in PAPSS2 are the cause of spondyloepimetaphyseal dysplasia Pakistani type

General

kinase or ATP-phosphotransferase phosphoprotein

Properties

SIZE: entity length = 614 aa MW = 70 kD MOTIF: Adenylyl-sulfate kinase {1} ATP-binding site NAME: ATP-binding site SITE: 49-56 serine residue {S123} kinase domain MOTIF: ATP-binding site NAME: ATP-binding site phosphorylation site PP {511-515} Sulfate adenylyltransferase {614}

Database Correlations

OMIM correlations MORBIDMAP 603005 UniProt O95340 PFAM correlations Entrez Gene 9060 Kegg hsa:9060 ENZYME correlations

References

UniProt :accession O95340