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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.
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| Protein type: EC 2.7.1.25; EC 2.7.7.4; Energy Metabolism - sulfur; Nucleotide Metabolism - purine; Other Amino Acids Metabolism - selenoamino acid; Transferase |
| Chromosomal Location of human Ortholog: 10q23.2-q23.31 |
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Cellular Component:
cytosol
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Molecular Function:
adenylylsulfate kinase activity; ATP binding; nucleotidyltransferase activity; protein binding; sulfate adenylyltransferase (ATP) activity
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Biological Process:
3'-phosphoadenosine 5'-phosphosulfate biosynthetic process; blood coagulation; bone development; hormone metabolic process; phosphorylation; sulfate assimilation
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Disease: Brachyolmia Type 4 With Mild Epiphyseal And Metaphyseal Changes
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Reference #:
O95340
(UniProtKB)
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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
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Gene Symbols: PAPSS2
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Molecular weight:
69,501 Da
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Basal Isoelectric point:
8.18
Predict pI for various phosphorylation states
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Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology®
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