Rao V L, Qureshi I A, Butterworth R F
Neuroscience Research Unit, André-Viallet Clinical Research Center (University of Montreal), Hopital Saint-Luc, Que., Canada.
Neurosci Lett. 1994 Mar 28;170(1):27-30. doi: 10.1016/0304-3940(94)90230-5.
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.
使用放射性测定法，在稀疏毛（spf/Y）小鼠的不同脑区测量了单胺氧化酶MAOA和MAOB的活性。spf/Y小鼠是一种由于鸟氨酸转氨甲酰酶的X染色体缺陷导致先天性高氨血症的模型。spf小鼠小脑（降低23%，P<0.05）和脑干（降低16%，P<0.05）中的MAOA活性降低；小脑（升高22%，P<0.05）、脑干（升高20%，P<0.05）和大脑皮层（升高22%，P<0.05）中的MAOB活性随之升高。这些发现为之前在spf小鼠大脑中发现单胺酸性代谢物增加的结果提供了合理的解释。单胺能功能改变可能是先天性高氨血症神经功能障碍发病机制中的一个关键因素。
