PAPSS1,2 transfer SO4(2-) group to ATP to form APS

Stable Identifier
Reaction [transition]
Homo sapiens
ATP + sulfate => adenylyl sulfate (APS) + pyrophosphate, Activation of inorganic sulfate forms APS
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
In the first step of PAPS biosynthesis, ATP and sulfate react to form adenylyl sulfate (APS) and pyrophosphate (PPi), catalyzed by the ATP sulfurylase domains of the bifunctional enzymes PAPS synthases 1 and 2 (PAPSS1 and 2). PAPSS2 is essential for the sulfation of glycosaminoglycan chains in proteoglycans, a necessary post translational modification. Defective PAPSS2 results in undersulfation of the glycosaminoglycan chains in proteoglycans which causes spondyloepimetaphyseal dysplasia Pakistani type (SEMD PA; MIM:612847), a bone disease characterized by epiphyseal dysplasia with mild metaphyseal abnormalities. Mutations resulting in SEMD PA include S438*, T48R and R329* (Ahmad et al. 1998, ul Haque et al. 1998, Noordam et al. 2009).
Literature References
PubMed ID Title Journal Year
9771708 Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse

Superti-Furga, A, Krakow, D, Ahmad, W, Cohn, DH, Rusiniak, ME, Haque, S, Abbas, H, King, LM, Ahmad, M, Swank, RT, ul Haque, MF, Cantor, RM

Nat Genet 1998
9576487 Sulfation in high endothelial venules: cloning and expression of the human PAPS synthetase

Girard, JP, Baekkevold, ES, Amalric, F

FASEB J 1998
9668121 Molecular cloning, expression, and characterization of human bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase and its functional domains

Venkatachalam, KV, Akita, H, Strott, CA

J Biol Chem 1998
Event Information
Catalyst Activity

sulfate adenylyltransferase (ATP) activity of PAPSS1,2 [cytosol]

Orthologous Events
Cross References
Cite Us!