PAPSS2 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. Clinical features include short stature evidenced at birth, short and bowed lower limbs, mild brachydactyly, kyphoscoliosis, abnormal gait, enlarged knee joints. Some patients may manifest premature pubarche and hyperandrogenism associated with skeletal dysplasia and short stature. 2 isoforms of the human protein are produced by alternative splicing. 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; metabolic process; phosphorylation; 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