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

More general terms

• kinase or ATP-phosphotransferase
• phosphoprotein

Additional terms

• 3'-phosphoadenosine-5'-phosphosulfate (PAPS, 3-phosphoadenylyl sulfate)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=9060
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:9060
• OMIM: https://mirror.omim.org/entry/603005
• OMIM: https://mirror.omim.org/entry/612847
• UniProt: http://www.uniprot.org/uniprot/O95340.html