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
Synonyms
adenylyl sulfate (APS) + ATP => PAPS + ADP, APS reacts with another ATP molecule 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
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
adenylylsulfate kinase activity of PAPSS1,2 [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Created