Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain

Adachi, 1972, Electron microscopic and enzyme histochemical studies of the cerebellum in spongy degeneration (van Bogaert and Bertrand type), Acta Neuropathol. (Berl.), 20, 22, 10.1007/BF00687899 Adachi, 1973, Spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan disease), Hum. Pathol., 4, 331, 10.1016/S0046-8177(73)80098-X Adornato, 1972, Cerebral spongy degeneration of infancy: a biochemical and ultrastructural study of affected twins, Neurology, 22, 202, 10.1212/WNL.22.2.202 Baslow, 1999, Expression of aspartoacylase activity in cultured rat macroglial cells is limited to oligodendrocytes, J. Mol. Neurosci., 13, 47, 10.1385/JMN:13:1-2:47 Berger, 1996, N-acetylated alpha linked acidic peptidase may be involved in axon–Schwann cell signaling, J. Neurocytol., 25, 499, 10.1007/BF02284818 Berger, 1999, Glutamate carboxypeptidase II is expressed by astrocytes in the adult rat nervous system, J. Comp. Neurol., 415, 52, 10.1002/(SICI)1096-9861(19991206)415:1<52::AID-CNE4>3.0.CO;2-K Birnbaum, 1955, Aminoacylases I and II from hog kidney, Methods Enzymol., 2, 115, 10.1016/S0076-6879(55)02176-9 Burlina, 1994, Detection of increased urinary N-acetylaspartylglutamate in Canavan disease, Eur. J. Pediatr., 153, 538, 10.1007/BF01957015 Burlina, 1999, N-acetylaspartylglutamate in Canavan disease: an adverse effector, Eur. J. Pediatr., 158, 406, 10.1007/s004310051102 Canavan, 1931, Schilder's encephalitis periaxialis diffusa, Arch. Neurol. Psychiatry, 25, 299, 10.1001/archneurpsyc.1931.02230020085005 Cassidy, 1993, N-acetylaspartylglutamate catabolism is achieved by an enzyme on the cell surface of neuron and glia, Neuropeptides, 24, 271, 10.1016/0143-4179(93)90015-3 Coyle, 1997, The nagging question of the function of N-acetylaspartylglutamate, Neurobiol. Dis., 4, 231, 10.1006/nbdi.1997.0153 Globus, 1928, Progressive degenerative subcortical encephalopathy (Schilder's disease), Arch. Neurol. Psychiatry, 20, 1190, 10.1001/archneurpsyc.1928.02210180041003 Jacobson, 1957, Studies on the role of N-acetylaspartic acid in mammalian brain, J. Gen. Physiol., 43, 323, 10.1085/jgp.43.2.323 Koller, 1984, N-acetyl-aspartyl-glutamate: regional levels in rat brain and the effects of brain lesions as determined by a new HPLC method, J. Neurochem., 43, 1136, 10.1111/j.1471-4159.1984.tb12854.x Matalon, 1988, Aspartoacylase deficiency N-acetylaspartic aciduria in patients with Canavan disease, Am. J. Med. Genet., 29, 463, 10.1002/ajmg.1320290234 Matalon, 1995, Canavan disease: from spongy degeneration to molecular analysis, J. Pediatr., 127, 511, 10.1016/S0022-3476(95)70105-2 Matalon, 2000, Knockout mouse for Canavan disease: a model for gene transfer to the central nervous system, J. Gene Med., 2, 165, 10.1002/(SICI)1521-2254(200005/06)2:3<165::AID-JGM107>3.0.CO;2-R Meyerhoff, 1992, Genetically epilepsy-prone rats have increased brain regional activity of an enzyme which liberates glutamate from N-acetyl-aspartyl-glutamate, Brain Res., 593, 140, 10.1016/0006-8993(92)91276-K Slusher, 1990, Rat brain N-acetylated α linked acidic dipeptidase activity: purification and immunologic characterization, J. Biol. Chem., 265, 21297, 10.1016/S0021-9258(17)45359-2 Surendran, 2003, Expression of glutamate transporter, GABRA6, serine proteinase inhibitor 2 and low levels of glutamate and GABA in the brain of knock-out mouse for Canavan disease, Brain Res. Bull., 61, 427, 10.1016/S0361-9230(03)00158-8 Surendran, 2003, Canavan disease: a monogenic trait with complex genomic interaction, Mol. Genet. Metab., 80, 74, 10.1016/j.ymgme.2003.08.015 Surendran, 2003, Metabolic changes in the knock-out mouse for canavan disease: implications to patients with CD, J. Child Neurol., 18, 611, 10.1177/08830738030180090701 Surendran, 2003, High levels of orexin A in the brain of the mouse model for phenylketonuria: possible role of orexin A in hyperactivity seen in children with PKU, Neurochem. Res., 28, 1891, 10.1023/A:1026184127689 Surendran, 2003, Mental retardation and hypotonia seen in the knock out mouse for Canavan disease is not due to succinate semialdehyde dehydrogenase deficiency, Neurosci. Lett., 358, 29, 10.1016/j.neulet.2003.12.105 Tallan, 1957, Studies on the distribution of N-acetyl-l-aspartic acid in brain, J. Biol. Chem., 224, 41, 10.1016/S0021-9258(18)65008-2 Tsai, 2003, Early embryonic death of glutamate carboxypeptidase II (NAALADase) homozygous mutants, Synapse, 50, 285, 10.1002/syn.10263 Tyson, 1998, Labeling of N-acetylaspartate and N-acetylaspartylglutamate in rat neocortex, hippocampus and cerebellum from [1−13C]glucose, Neurosci. Lett., 251, 181, 10.1016/S0304-3940(98)00527-8 van Bogert, 1949, Sur une idiotie familial avec degenerescence sponglieuse de neurax (note preliminaire), Acta Neurol. Belg., 49, 572