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: 10q23.2-q23.31
Cellular Component:  cytosol
Molecular Function:  ATP binding; nucleotidyltransferase activity; sulfate adenylyltransferase (ATP) activity
Biological Process:  3'-phosphoadenosine 5'-phosphosulfate biosynthetic process; blood coagulation; bone development; phosphorylation; skeletal system development; sulfate assimilation
Disease: Brachyolmia Type 4 With Mild Epiphyseal And Metaphyseal Changes
Reference #:  O95340 (UniProtKB)
Alt. Names/Synonyms: 3'-phosphoadenosine 5'-phosphosulfate synthase 2; 3'-phosphoadenosine-5'-phosphosulfate synthase; 3-prime-phosphoadenosine 5-prime-phosphosulfate synthase 2; adenosine 5'-phosphosulfate kinase; Adenosine-5'-phosphosulfate 3'-phosphotransferase; Adenylyl-sulfate kinase; Adenylylsulfate 3'-phosphotransferase; APS kinase; ATP sulfurylase/adenosine 5'-phosphosulfate kinase; ATP sulfurylase/APS kinase 2; ATP-sulfurylase; ATPSK2; BCYM4; Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2; bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthethase 2; epididymis secretory sperm binding protein; PAPS synthase 2; PAPS synthetase 2; PAPS2; PAPSS 2; PAPSS2; SAT; SK 2; SK2; Sulfate adenylate transferase; Sulfate adenylyltransferase; Sulfurylase kinase 2
Gene Symbols: PAPSS2
Molecular weight: 69,501 Da
Basal Isoelectric point: 8.18  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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