PAPSS2 a 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. May have a important role in skeletogenesis during postnatal growth. Defects in PAPSS2 are the cause of spondyloepimetaphyseal dysplasia Pakistani type (SEMD-PA). A bone disease characterized by epiphyseal dysplasia with mild metaphyseal abnormalities. The N-terminal section belongs to the adenylyl-sulfate kinase (APS) family. The C-terminal section belongs to the sulfate adenylyltransferase family. Expressed in cartilage and adrenal gland. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; EC; Energy Metabolism - sulfur; Nucleotide Metabolism - purine; Other Amino Acids Metabolism - selenoamino acid; Transferase
Chromosomal Location of Human Ortholog: 19 C1|19 27.46 cM
Cellular Component: 
Molecular Function:  adenylylsulfate kinase activity; ATP binding; catalytic activity; kinase activity; nucleotide binding; nucleotidyltransferase activity; sulfate adenylyltransferase (ATP) activity; transferase activity
Biological Process:  3'-phosphoadenosine 5'-phosphosulfate biosynthetic process; blood coagulation; bone development; metabolic process; sulfate assimilation
Reference #:  O88428 (UniProtKB)
Alt. Names/Synonyms: 1810018P12Rik; 3'-phosphoadenosine 5'-phosphosulfate synthase 2; 3'-phosphoadenosine-5'-phosphosulfate synthase; Adenosine-5'-phosphosulfate 3'-phosphotransferase; Adenylyl-sulfate kinase; Adenylylsulfate 3'-phosphotransferase; AI159688; APS kinase; ATP-sulfurylase; Atpsk2; AtpsU2; Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2; bm; brachymorphic; code642; PAPS synthase 2; PAPS2; PAPSS 2; Papss2; SAT; SK 2; SK2; Sulfate adenylate transferase; Sulfate adenylyltransferase; Sulfurylase kinase 2
Gene Symbols: Papss2
Molecular weight: 70,351 Da
Basal Isoelectric point: 7.31  Predict pI for various phosphorylation states
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Protein Structure Not Found.

Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene