ASPA deacetylates NAA to acetate and L-aspartate

Stable Identifier
R-HSA-5691507
Type
Reaction
Species
Homo sapiens
Compartment
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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
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Catalyst Activity

aspartoacylase activity of ASPA:Zn2+ dimer [cytosol]

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Rhea
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