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Gene Review

ASPA  -  aspartoacylase

Homo sapiens

Synonyms: ACY-2, ACY2, ASP, Aminoacylase-2, Aspartoacylase
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Disease relevance of ASPA


High impact information on ASPA


Chemical compound and disease context of ASPA

  • Our success in preparing the recombinant ASPA in high purity should permit multiple lines of investigations to understand the pathogenic mechanisms of Canavan disease and the functional roles of NAA [8].

Biological context of ASPA


Anatomical context of ASPA


Associations of ASPA with chemical compounds

  • Aspartoacylase catalyzes the deacetylation of N-acetylaspartic acid (NAA) in the brain to produce acetate and L-aspartate [3].
  • A description is give of the effect on the ASPA cement reaction of tartaric acid incorporated in the cement liquid [17].
  • Our hypothesis is that ASPA actively participates in myelin synthesis by providing NAA-derived acetate for acetyl CoA synthesis, which in turn is used for synthesis of the lipid portion of myelin [18].
  • The retentive capacity of the Whaledent Parapost system utilizing ASPA cement was compared at two lengths, three diameters, and with respect to the effect of both medication and citric acid pretreatment [19].
  • The release of fluoride from a glass ionomer cement (ASPA) was compared with that from a silicate cement [20].

Other interactions of ASPA


Analytical, diagnostic and therapeutic context of ASPA


  1. Identification and expression of eight novel mutations among non-Jewish patients with Canavan disease. Kaul, R., Gao, G.P., Matalon, R., Aloya, M., Su, Q., Jin, M., Johnson, A.B., Schutgens, R.B., Clarke, J.T. Am. J. Hum. Genet. (1996) [Pubmed]
  2. The molecular basis of canavan (aspartoacylase deficiency) disease in European non-Jewish patients. Shaag, A., Anikster, Y., Christensen, E., Glustein, J.Z., Fois, A., Michelakakis, H., Nigro, F., Pronicka, E., Ribes, A., Zabot, M.T. Am. J. Hum. Genet. (1995) [Pubmed]
  3. Purification and preliminary characterization of brain aspartoacylase. Moore, R.A., Le Coq, J., Faehnle, C.R., Viola, R.E. Arch. Biochem. Biophys. (2003) [Pubmed]
  4. Aspartoacylase gene transfer to the mammalian central nervous system with therapeutic implications for Canavan disease. Leone, P., Janson, C.G., Bilaniuk, L., Wang, Z., Sorgi, F., Huang, L., Matalon, R., Kaul, R., Zeng, Z., Freese, A., McPhee, S.W., Mee, E., During, M.J., Bilianuk, L. Ann. Neurol. (2000) [Pubmed]
  5. Localization of an open reading frame with homology to human aspartoacylase upstream from psbA in the prokaryote Prochlorococcus marinus CCMP 1375. Hess, W.R. DNA Seq. (1997) [Pubmed]
  6. From the Cover: Structure of aspartoacylase, the brain enzyme impaired in Canavan disease. Bitto, E., Bingman, C.A., Wesenberg, G.E., McCoy, J.G., Phillips, G.N. Proc. Natl. Acad. Sci. U.S.A. (2007) [Pubmed]
  7. Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease. Madhavarao, C.N., Arun, P., Moffett, J.R., Szucs, S., Surendran, S., Matalon, R., Garbern, J., Hristova, D., Johnson, A., Jiang, W., Namboodiri, M.A. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  8. Murine aspartoacylase: cloning, expression and comparison with the human enzyme. Namboodiri, M.A., Corigliano-Murphy, A., Jiang, G., Rollag, M., Provencio, I. Brain Res. Mol. Brain Res. (2000) [Pubmed]
  9. Mild-onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase gene. Janson, C.G., Kolodny, E.H., Zeng, B.J., Raghavan, S., Pastores, G., Torres, P., Assadi, M., McPhee, S., Goldfarb, O., Saslow, B., Freese, A., Wang, D.J., Bilaniuk, L., Shera, D., Leone, P. Ann. Neurol. (2006) [Pubmed]
  10. A benign polymorphism in the aspartoacylase gene may cause misinterpretation of Canavan gene testing. Propheta, O., Magal, N., Shohat, M., Eyal, N., Navot, N., Horowitz, M. Eur. J. Hum. Genet. (1998) [Pubmed]
  11. Two novel aspartoacylase gene (ASPA) missense mutations specific to Norwegian and Swedish patients with Canavan disease. Olsen, T.R., Tranebjaerg, L., Kvittingen, E.A., Hagenfeldt, L., Møller, C., Nilssen, O. J. Med. Genet. (2002) [Pubmed]
  12. Identification and characterization of novel mutations of the aspartoacylase gene in non-Jewish patients with Canavan disease. Zeng, B.J., Wang, Z.H., Ribeiro, L.A., Leone, P., De Gasperi, R., Kim, S.J., Raghavan, S., Ong, E., Pastores, G.M., Kolodny, E.H. J. Inherit. Metab. Dis. (2002) [Pubmed]
  13. N-acetylaspartic aciduria due to aspartoacylase deficiency--a new aetiology of childhood leukodystrophy. Hagenfeldt, L., Bollgren, I., Venizelos, N. J. Inherit. Metab. Dis. (1987) [Pubmed]
  14. Canavan disease. Analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms. Baslow, M.H., Resnik, T.R. J. Mol. Neurosci. (1997) [Pubmed]
  15. Adeno-associated virus-mediated aspartoacylase gene transfer to the brain of knockout mouse for canavan disease. Matalon, R., Surendran, S., Rady, P.L., Quast, M.J., Campbell, G.A., Matalon, K.M., Tyring, S.K., Wei, J., Peden, C.S., Ezell, E.L., Muzyczka, N., Mandel, R.J. Mol. Ther. (2003) [Pubmed]
  16. Adenoviral gene transfer of aspartoacylase ameliorates tonic convulsions of spontaneously epileptic rats. Seki, T., Matsubayashi, H., Amano, T., Kitada, K., Serikawa, T., Sasa, M., Sakai, N. Neurochem. Int. (2004) [Pubmed]
  17. Reactions in glass ionomer cements: V. Effect of incorporating tartaric acid in the cement liquid. Crisp, S., Wilson, A.D. J. Dent. Res. (1976) [Pubmed]
  18. Canavan disease and the role of N-acetylaspartate in myelin synthesis. Namboodiri, A.M., Peethambaran, A., Mathew, R., Sambhu, P.A., Hershfield, J., Moffett, J.R., Madhavarao, C.N. Mol. Cell. Endocrinol. (2006) [Pubmed]
  19. Dowel retention with glass-ionomer cement. Krupp, J.D., Caputo, A.A., Trabert, K.C., Standlee, J.P. The Journal of prosthetic dentistry. (1979) [Pubmed]
  20. Fluoride release from a glass ionomer cement. Forsten, L. Scandinavian journal of dental research. (1977) [Pubmed]
  21. Polymorphism of the cobalt-activated acylase in human tissues. Ziomek, E., Szewczuk, A. Acta Biochim. Pol. (1978) [Pubmed]
  22. Human platelet antigen-5 (Br) genotyping by ASPA: allele-specific primer amplification (PCR-SSP). Simsek, S., Bleeker, P.M., Heeremans, J., von dem Borne, A.E. Br. J. Haematol. (1994) [Pubmed]
  23. Aspartoacylase is a regulated nuclear-cytoplasmic enzyme. Hershfield, J.R., Madhavarao, C.N., Moffett, J.R., Benjamins, J.A., Garbern, J.Y., Namboodiri, A. FASEB J. (2006) [Pubmed]
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