ID:PAPS2_HUMAN DESCRIPTION: RecName: Full=Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2; Short=PAPS synthase 2; Short=PAPSS 2; AltName: Full=Sulfurylase kinase 2; Short=SK 2; Short=SK2; Includes: RecName: Full=Sulfate adenylyltransferase; EC=2.7.7.4; AltName: Full=ATP-sulfurylase; AltName: Full=Sulfate adenylate transferase; Short=SAT; Includes: RecName: Full=Adenylyl-sulfate kinase; EC=2.7.1.25; AltName: Full=3'-phosphoadenosine-5'-phosphosulfate synthase; AltName: Full=APS kinase; AltName: Full=Adenosine-5'-phosphosulfate 3'-phosphotransferase; AltName: Full=Adenylylsulfate 3'-phosphotransferase; FUNCTION: 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 (By similarity). CATALYTIC ACTIVITY: ATP + sulfate = diphosphate + adenylyl sulfate. CATALYTIC ACTIVITY: ATP + adenylyl sulfate = ADP + 3'- phosphoadenylyl sulfate. PATHWAY: Sulfur metabolism; sulfate assimilation. TISSUE SPECIFICITY: Expressed in cartilage and adrenal gland. DISEASE: Defects in PAPSS2 are the cause of spondyloepimetaphyseal dysplasia Pakistani type (SEMD-PA) [MIM:612847]. 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. SIMILARITY: In the N-terminal section; belongs to the APS kinase family. SIMILARITY: In the C-terminal section; belongs to the sulfate adenylyltransferase family.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on O95340
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
BC009894 - Homo sapiens 3'-phosphoadenosine 5'-phosphosulfate synthase 2, mRNA (cDNA clone MGC:1916 IMAGE:2988345), complete cds. GQ891452 - Homo sapiens clone HEL-S-173 epididymis secretory sperm binding protein mRNA, complete cds. AK312556 - Homo sapiens cDNA, FLJ92929, highly similar to Homo sapiens 3'-phosphoadenosine 5'-phosphosulfate synthase 2 (PAPSS2), mRNA. AF091242 - Homo sapiens ATP sulfurylase/APS kinase 2 mRNA, complete cds. AF150754 - Homo sapiens 3'phosphoadenosine 5'-phosphosulfate synthase 2b isoform mRNA, complete cds. JD272536 - Sequence 253560 from Patent EP1572962. JD272537 - Sequence 253561 from Patent EP1572962. JD200351 - Sequence 181375 from Patent EP1572962. JD200352 - Sequence 181376 from Patent EP1572962. AF074331 - Homo sapiens PAPS synthetase-2 (PAPSS2) mRNA, complete cds. JD423308 - Sequence 404332 from Patent EP1572962. AF173365 - Homo sapiens ATP sulfurylase/APS kinase isoform SK2 mRNA, alternatively spliced, complete cds. DQ893625 - Synthetic construct clone IMAGE:100006255; FLH184327.01X; RZPDo839E09144D 3'-phosphoadenosine 5'-phosphosulfate synthase 2 (PAPSS2) gene, encodes complete protein. DQ895430 - Synthetic construct Homo sapiens clone IMAGE:100009890; FLH184323.01L; RZPDo839E09143D 3'-phosphoadenosine 5'-phosphosulfate synthase 2 (PAPSS2) gene, encodes complete protein. KJ905366 - Synthetic construct Homo sapiens clone ccsbBroadEn_14924 PAPSS2 gene, encodes complete protein. KR710243 - Synthetic construct Homo sapiens clone CCSBHm_00010845 PAPSS2 (PAPSS2) mRNA, encodes complete protein. KR710244 - Synthetic construct Homo sapiens clone CCSBHm_00010846 PAPSS2 (PAPSS2) mRNA, encodes complete protein. KJ892677 - Synthetic construct Homo sapiens clone ccsbBroadEn_02071 PAPSS2 gene, encodes complete protein. AF313907 - Homo sapiens 3'-phosphoadenosine 5'-phosphosulfate synthase 2 alpha mRNA, complete cds. AK301453 - Homo sapiens cDNA FLJ53931 complete cds, highly similar to Bifunctional 3'-phosphoadenosine5'-phosphosulfate synthetase 2. AK309262 - Homo sapiens cDNA, FLJ99303. JD487390 - Sequence 468414 from Patent EP1572962.
Biochemical and Signaling Pathways
BioCyc Knowledge Library PWY-5340 - sulfate activation for sulfonation
Reactome (by CSHL, EBI, and GO)
Protein O95340 (Reactome details) participates in the following event(s):
R-HSA-174389 PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS R-HSA-174392 PAPSS1,2 transfer SO4(2-) group to ATP to form APS R-HSA-2408525 H2SeO4 is converted to APSe by PAPSS1,2 R-HSA-2408540 APSe is phosphorylated to PAPSe by PAPSS1,2 R-HSA-174362 Transport and synthesis of PAPS R-HSA-2408550 Metabolism of ingested H2SeO4 and H2SeO3 into H2Se R-HSA-156584 Cytosolic sulfonation of small molecules R-HSA-1630316 Glycosaminoglycan metabolism R-HSA-2408522 Selenoamino acid metabolism R-HSA-156580 Phase II - Conjugation of compounds R-HSA-71387 Metabolism of carbohydrates R-HSA-71291 Metabolism of nitrogenous molecules R-HSA-211859 Biological oxidations R-HSA-1430728 Metabolism
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
