Reactome | PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS

PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS

Stable Identifier

R-HSA-174389

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

adenylyl sulfate (APS) + ATP => PAPS + ADP, APS reacts with another ATP molecule to form PAPS

ReviewStatus

5/5

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PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS

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In the second step of PAPS biosynthesis, adenylyl sulfate (APS) is phosphorylated to 3'-phosphoadenylyl sulfate (PAPS), catalyzed by the APS kinase domains of the bifunctional enzymes PAPS synthases 1 and 2 (PAPSS1 and 2). PAPSS2 is essential for the sulfation of glycosaminoglycan chains of proteoglycans, a necessary post-translational modification. Defective PAPSS2 results in undersulfation of 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
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
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 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