GLUTAMATE AND GAMMA-AMINOBUTYRIC-ACID NEUROTRANSMITTER SYSTEMS IN THE ACUTE PHASE OF MAPLE SYRUP URINE DISEASE AND CITRULLINEMIA ENCEPHALOPATHIES IN NEWBORN CALVES
GLUTAMATE AND GAMMA-AMINOBUTYRIC-ACID NEUROTRANSMITTER SYSTEMS IN THE ACUTE PHASE OF MAPLE SYRUP URINE DISEASE AND CITRULLINEMIA ENCEPHALOPATHIES IN NEWBORN CALVES
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DOI：
10.1111/j.1471-4159.1992.tb09409.x
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发表时间：
1992-08-01
影响因子：
4.7
通讯作者：
JOHNSTON, GAR
中科院分区：
文献类型：
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作者：
DODD, PR;WILLIAMS, SH;JOHNSTON, GAR
尸检时，从患有临床确诊的枫糖浆尿病（MSUD）的新生无角赫里福德犊牛、患有临床确诊的瓜氨酸血症的新生荷斯坦-弗里斯兰犊牛和匹配对照组中获得大脑皮质组织。由此，突触体制备的神经递质氨基酸的摄取和刺激诱导的释放的研究，和突触质膜获得相关的突触后受体结合位点的研究。除了具有异常的脑组织病理学血浆氨基酸浓度（MSUD中支链化合物缬氨酸、异亮氨酸和亮氨酸的水平显著增加;瓜氨酸血症中瓜氨酸水平显著升高）外，两组患病动物均显示出每种递质氨基酸谷氨酸、天冬氨酸和γ-氨基丁酸（GABA）的脑组织浓度降低。在这两种疾病中，非递质氨基酸一般不受影响。瓜氨酸血症小牛的脑谷氨酰胺浓度显著增加;在MSUD小牛中，谷氨酰胺浓度升高，但程度要小得多。Na+依赖性突触体对谷氨酸和GABA的摄取明显减少（至
Cerebral cortex tissue was obtained at autopsy from neonatal Poll Hereford calves with clinically confirmed maple syrup urine disease (MSUD), neonatal Holstein-Friesian calves with clinically confirmed citrullinemia, and matched controls. From this, synaptosomes were prepared for studies of neurotransmitter amino acid uptake and stimulus-induced release, and synaptic plasma membranes were obtained for studies of associated postsynaptic receptor binding sites. As well as having abnormal brain tissue concentrations of the pathognomic plasma amino acids (markedly increased levels of the branched-chain compounds valine, isoleucine, and leucine in MSUD; marked elevation of citrulline levels in citrullinemia), both groups of diseased animals showed reduced brain tissue concentrations of each of the transmitter amino acids glutamate, aspartate, and gamma-aminobutyric acid (GABA). Nontransmitter amino acids were generally unaffected in either disease. Citrullinemic calves showed a marked increase in brain glutamine concentration; in calves with MSUD, the glutamine concentration was raised, but to a much lesser extent. The Na+-dependent synaptosomal uptake of both glutamate and GABA was markedly reduced (to