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

ReviewStatus

5/5

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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
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