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

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

Stable Identifier

R-HSA-174392

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

ATP + sulfate => adenylyl sulfate (APS) + pyrophosphate, Activation of inorganic sulfate forms APS

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

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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
9576487	Sulfation in high endothelial venules: cloning and expression of the human PAPS synthetase Girard, JP, Baekkevold, ES, Amalric, F	FASEB J	1998
9771708	Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse 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	Nat Genet	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