Reactome | ASPA deacetylates NAA to acetate and L-aspartate

ASPA deacetylates NAA to acetate and L-aspartate

Stable Identifier

R-HSA-5691507

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

5/5

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ASPA deacetylates NAA to acetate and L-aspartate

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Aspartoacylase (ASPA) is a cytosolic zinc metalloenzyme highly expressed in brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver. ASPA catalyses the hydrolysis of N-acetylaspartic acid (NAA) to produce acetate (CH3COO-) and L-aspartate (L-Asp). NAA occurs in high concentration in brain and is thought to play a significant part in the maintenance of intact white matter. In other tissues it acts as a scavenger of NAA from body fluids. Defects in ASPA lead to Canavan disease (CAND; MIM:271900), a fatal neurological disorder of infants characterised by white matter vacuolisation and demyelination (Herga et al. 2006, Le Coq et al. 2006, Bitto et al. 2007).

Literature References

PubMed ID	Title	Journal	Year
17027983	Identification of the zinc binding ligands and the catalytic residue in human aspartoacylase, an enzyme involved in Canavan disease Berrin, JG, Giardina, T, Perrier, J, Herga, S, Puigserver, A	FEBS Lett.	2006
16669630	Characterization of human aspartoacylase: the brain enzyme responsible for Canavan disease Lebrilla, C, An, HJ, Le Coq, J, Viola, RE	Biochemistry	2006
17194761	Structure of aspartoacylase, the brain enzyme impaired in Canavan disease Bingman, CA, Bitto, E, Phillips, GN, McCoy, JG, Wesenberg, GE	Proc. Natl. Acad. Sci. U.S.A.	2007