Dienel Gerald A, Cruz Nancy F
Department of Neurology, University of Arkansas for Medical Sciences, Slot 500, 4301 W. Markham St., Shorey Bldg., Room 715, Little Rock, AR, 72205, USA,
Metab Brain Dis. 2014 Dec;29(4):1041-52. doi: 10.1007/s11011-013-9442-y. Epub 2013 Oct 25.
Portacaval shunting is a model for hepatic encephalopathy that causes chronic hyperammonemia, disruption of metabolic, signaling, and neurotransmitter systems, and progressive morphological changes. Exposure of cultured cells to ammonia raises intralysosomal pH and inhibits proteolysis, and the present study tested the hypothesis that proteolytic capacity is diminished in portacaval-shunted rats. Proteins were labeled in vivo with tracer doses of diisopropylfluorophosphate (DFP) and clearance of label was assayed. This approach labeled proteins independent of protein synthesis, which is reported to be altered in shunted rats, and avoided complications arising from re-utilization of labeled amino acids that causes underestimation of degradation rate. Characterization of DFP labeling showed that protein labeling was fast, about 50% of the label was released during a 24 h interval, labeling by DFP metabolites was negligible, inhibition of brain acetylcholinesterase was not detectable, and labeling by [(3)H]- and [(14)C]DFP was equivalent. To assay degradative capacity, proteins were first labeled with [(3)H]DFP, followed by labeling with [(14)C]DFP that was given 24 or 72 h later. The (3)H/(14)C ratio in each animal was used as a relative measure of removal of (3)H-labeled proteins. (3)H/(14)C ratios were generally significantly higher in portacaval-shunted rats than in controls, consistent with reduced proteolytic capacity. Assays of amino acid incorporation into brain protein generally replicated literature reports, supporting the conclusion that protein synthesis unlikely to be markedly inhibited and amino acid recycling influences calculated protein synthesis rates in shunted rats. Therapeutic strategies to reduce ammonia level would help normalize lysosomal functions and protein and lipid turnover.
门腔分流术是一种肝性脑病模型,可导致慢性高氨血症、代谢、信号传导和神经递质系统紊乱以及进行性形态学改变。将培养的细胞暴露于氨中会升高溶酶体内pH值并抑制蛋白水解,本研究检验了门腔分流大鼠蛋白水解能力降低的假说。用示踪剂量的二异丙基氟磷酸(DFP)在体内标记蛋白质,并测定标记物的清除率。这种方法标记蛋白质与蛋白质合成无关,据报道分流大鼠的蛋白质合成会发生改变,并且避免了因标记氨基酸再利用导致降解率低估而产生的并发症。DFP标记的特征表明,蛋白质标记迅速,在24小时内约50%的标记物被释放,DFP代谢产物的标记可忽略不计,未检测到对脑乙酰胆碱酯酶的抑制,并且[(3)H]-和[(14)C]DFP的标记效果相当。为了测定降解能力,首先用[(3)H]DFP标记蛋白质,然后在24或72小时后用[(14)C]DFP标记。每只动物的(3)H/(14)C比值用作去除(3)H标记蛋白质的相对指标。门腔分流大鼠的(3)H/(14)C比值通常显著高于对照组,这与蛋白水解能力降低一致。氨基酸掺入脑蛋白的测定通常重复了文献报道,支持了蛋白质合成不太可能被显著抑制且氨基酸再循环影响分流大鼠计算出的蛋白质合成速率这一结论。降低氨水平的治疗策略将有助于使溶酶体功能以及蛋白质和脂质周转正常化。