title: Activities of monoamine oxidase-A and -B are altered in the brains of congenitally hyperammonemic sparse-fur (spf) mice.
abstract: Activities of monoamine oxidases, MAOA and MAOB, were measured using radiometric assays in different brain regions of the sparse-fur (spf/Y) mouse, a model of congenital hyperammonemia resulting from an X-chromosomal defect of ornithine transcarbamylase. MAOA activities were decreased in cerebellum (by 23%, P < 0.05) and brainstem (by 16%, P < 0.05) of spf mice; activities of MAOB were concomitantly increased in cerebellum (by 22%, P < 0.05), brainstem (by 20%, P < 0.05) and cerebral cortex (by 22%, P < 0.05). These findings offer a rational explanation for previous findings of increased acidic metabolites of monoamines in the brain of spf mice. Altered monoaminergic function could be a key factor in the pathogenesis of neurological dysfunction in congenital hyperammonemias.
authors.affiliation: Neuroscience Research Unit, André-Viallet Clinical Research Center (University of Montreal), Hopital Saint-Luc, Que., Canada.
authors.forename: V L
authors.initials: VL
authors.lastname: Rao
authors.forename: I A
authors.initials: IA
authors.lastname: Qureshi
authors.forename: R F
authors.initials: RF
authors.lastname: Butterworth
mesh_terms: D000641:Ammonia / Q000097:blood*; D000818:Animals; D001921:Brain / Q000201:enzymology*; D001923:Brain Chemistry / Q000502:physiology; D008297:Male; D008661:Metabolism, Inborn Errors / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D008818:Mice, Neurologic Mutants; D008995:Monoamine Oxidase / Q000378:metabolism*; D009954:Ornithine Carbamoyltransferase / Q000235:genetics / Q000378:metabolism; D012701:Serotonin / Q000378:metabolism
chemical_list: D012701:Serotonin; D000641:Ammonia; D008995:Monoamine Oxidase; D009954:Ornithine Carbamoyltransferase