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Iranian Journal of Child Neurology, ISSN 1735-4668, 2015, Volume 9, Issue 4, pp. 54 - 57
Objective Canavan disease (CD) is a type of vacuolating leukodystrophy with autosomal recessive inheritance. Aspartoacylase deficiency results in decrease of... 
ASPA gene | Canavan disease | Aspartoacylase deficiency | Aspartoacylase enzyme | Case Report
Journal Article
Journal Article
Acta Neuropathologica, ISSN 0001-6322, 1/2018, Volume 135, Issue 1, pp. 95 - 113
Journal Article
Journal Article
Experimental Animals, ISSN 1341-1357, 2016
Essential tremor (ET) is a common movement disorder with a poorly understood etiology. The TRM/Kyo mutant rat, showing spontaneous tremor, is an animal model... 
aspartoacylase | rat | TALEN | essential tremor | Hcn1
Journal Article
Journal Article
Journal Article
Brain Research, ISSN 0006-8993, 09/2019, Volume 1718, pp. 169 - 175
The rat is an autosomal recessive mutant that exhibits severe rapid myelin breakdown throughout the central nervous system at 7–8 weeks of age. The rat has a... 
Aspartoacylase | Mitochondria | Mutant rat | Oligodendrocyte | Dysmyelination | ASPARTATE | PATHOLOGY | MODEL | NEUROSCIENCES | OLIGODENDROCYTES | TAIEP RAT | TREMOR | N-ACETYLASPARTATE | ACCUMULATION | EXPRESSION | BRAIN | Enzymes | Metabolites | RNA | Gene mutations | Mass spectrometry
Journal Article
Frontiers in Molecular Neuroscience, ISSN 1662-5099, 06/2017, Volume 10, pp. 161 - 161
Canavan disease is caused by mutations in the gene encoding aspartoacylase (ASPA), a deacetylase that catabolizes N-acetylaspartate (NAA). The precise... 
Vacuolization | Acss2 | Aspartoacylase | Acss1 | NAAG | Nat8L | Vacuoles | NAA | ACETYL-L-ASPARTATE | LIPID-SYNTHESIS | vacuolization | BROWN ADIPOCYTES | ANTIOXIDANT DEFENSES | NEUROSCIENCES | ASPARTOACYLASE GENE | aspartoacylase | vacuoles | SPONGY DEGENERATION | MOUSE MODEL | TREMOR RAT | CENTRAL-NERVOUS-SYSTEM | RAT-BRAIN
Journal Article
JOURNAL OF NEUROSCIENCE, ISSN 0270-6474, 01/2017, Volume 37, Issue 2, pp. 413 - 421
Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves... 
myelin | HUMAN BRAIN | WHITE-MATTER | N-acetyl-L-aspartate | SYSTEM VAN-BOGAERT | MOUSE | neuron | N-acetyltransferase-8-like | ASTROCYTES | NEUROSCIENCES | MYELIN LIPID-SYNTHESIS | Canavan disease | aspartoacylase | SPONGY DEGENERATION | IN-VIVO | DISEASE | ACETYLASPARTATE
Journal Article