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3'-phosphoadenosine 5'-phosphosulfate synthase 2
Protein PAPSS2 PDB 2ax4.png
PDB rendering based on 2ax4.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols PAPSS2 ; ATPSK2; BCYM4; SK2
External IDs OMIM603005 MGI1330223 HomoloGene55840 GeneCards: PAPSS2 Gene
EC number,
RNA expression pattern
PBB GE PAPSS2 203058 s at tn.png
PBB GE PAPSS2 203059 s at tn.png
PBB GE PAPSS2 203060 s at tn.png
More reference expression data
Species Human Mouse
Entrez 9060 23972
Ensembl ENSG00000198682 ENSMUSG00000024899
UniProt O95340 O88428
RefSeq (mRNA) NM_001015880 NM_001201470
RefSeq (protein) NP_001015880 NP_001188399
Location (UCSC) Chr 10:
89.42 – 89.51 Mb
Chr 19:
32.62 – 32.67 Mb
PubMed search [1] [2]

Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetase 2 is an enzyme that in humans is encoded by the PAPSS2 gene.[1][2]

Sulfation is a common modification of endogenous (lipids, proteins, and carbohydrates) and exogenous (xenobiotics and drugs) compounds. In mammals, the sulfate source is 3'-phosphoadenosine 5'-phosphosulfate (PAPS), created from ATP and inorganic sulfate. Two different tissue isoforms encoded by different genes synthesize PAPS. This gene encodes one of the two PAPS synthetases. Defects in this gene cause the Pakistani type of spondyloepimetaphyseal dysplasia. Two alternatively spliced transcript variants that encode different isoforms have been described for this gene.[2]


  1. ^ ul Haque MF, King LM, Krakow D, Cantor RM, Rusiniak ME, Swank RT, Superti-Furga A, Haque S, Abbas H, Ahmad W, Ahmad M, Cohn DH (Oct 1998). "Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse". Nat Genet 20 (2): 157–62. doi:10.1038/2458. PMID 9771708. 
  2. ^ a b "Entrez Gene: PAPSS2 3'-phosphoadenosine 5'-phosphosulfate synthase 2". 

Further reading[edit]