Transport and synthesis of PAPS

Stable Identifier
Homo sapiens
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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
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