Reactome | Transport and synthesis of PAPS

Transport and synthesis of PAPS

Stable Identifier

R-HSA-174362

DOI

10.3180/R-HSA-174362.3

Type

Pathway

Species

Homo sapiens

ReviewStatus

5/5

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PAPS (3'-phosphoadenosine-5'-phosphosulfate), which functions as a sulfate donor in the cell, is synthesized from sulfate and two molecules of ATP in a two-step process (Robbins & Lipmann 1958) catalyzed in vertebrates by a bifunctional enzyme (Venkatachalam et al. 1998). PAPS synthesis takes place in the cytosol, and it is either consumed there in the sulfonation of a variety of hormones and xenobiotics, or it is transported to the Golgi apparatus and consumed in the synthesis of proteoglycans like chondroitin sulfate. Two isoforms of the human bifunctional enzyme are known, mutations in one of which are associated with defects in proteoglycan biosynthesis (Girard et al. 1998, ul Haque et al. 1998).

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
13575436	Separation of the two enzymatic phases in active sulfate synthesis Lipmann, F, Robbins, PW	J Biol Chem	1958