Literature summary for 3.5.1.15 extracted from

Klugmann, M.; Leichtlein, C.B.; Symes, C.W.; Serikawa, T.; Young, D.; During, M.J.
Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease (2005), Mol. Ther., 11, 745-753.

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Cloned(Commentary)

Cloned (Comment)	Organism
-	Homo sapiens

Protein Variants

Protein Variants	Comment	Organism
additional information	restoration of deficient enzyme activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease, which is caused by elevated levels of N-acetylaspartic acid, NAA, neuronal expression of ASPA can compensate for NAA-mediated neuronal hyperexcitation, but not for oligodebdrocyte dysfunciton, phenotypes, overview	Rattus norvegicus

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
cytosol	-	Homo sapiens	5829	-
cytosol	-	Rattus norvegicus	5829	-

Molecular Weight [Da]

Molecular Weight [Da]	Molecular Weight Maximum [Da]	Comment	Organism
37000	-	determined by SDS-PAGE	Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
N-acetylaspartate + H2O	Homo sapiens	aspartocylase deficiency results in elevated levels of substrate, brain edema and dysmyelination	L-aspartate + acetate	-	?
N-acetylaspartic acid + H2O	Rattus norvegicus	restoration of deficient enzyme activity by enzyme expression in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease, which is caused by elevated levels of N-acetylaspartic acid, NAA, neuronal expression of ASPA can compensate for NAA-mediated neuronal hyperexcitation, but not for oligodebdrocyte dysfunciton, overview	L-asparatate + acetate	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-
Rattus norvegicus	-	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
brain	-	Rattus norvegicus	-
neuron	-	Homo sapiens	-
neuron	-	Rattus norvegicus	-
oligodendrocyte	-	Rattus norvegicus	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
N-acetylaspartate + H2O	aspartocylase deficiency results in elevated levels of substrate, brain edema and dysmyelination	Homo sapiens	L-aspartate + acetate	-	?
N-acetylaspartic acid + H2O	-	Rattus norvegicus	L-asparatate + acetate	-	?
N-acetylaspartic acid + H2O	restoration of deficient enzyme activity by enzyme expression in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease, which is caused by elevated levels of N-acetylaspartic acid, NAA, neuronal expression of ASPA can compensate for NAA-mediated neuronal hyperexcitation, but not for oligodebdrocyte dysfunciton, overview	Rattus norvegicus	L-asparatate + acetate	-	?

Synonyms

Synonyms	Comment	Organism
ASPA	-	Rattus norvegicus
Aspartoacylase	-	Homo sapiens

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Release 2023.1 (January 2023)