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N-acetylamino acid utilization by kidney aminoacylase-1

机译:肾脏氨酰酶-1对N-乙酰氨基酸的利用

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摘要

Mammalian aminoacylase-1 (Acy1) participates in the breakdown of N-acetylated amino acids during intracellular protein catabolism. Acy 1 is most abundantly expressed in the kidney tubular epithelium. Lately, Acy1 deficiency was identified in children with increased urinary excretion of several N-acetylamino acids. Here we report detailed N-acetylamino acid specificity profiles for human and porcine Acy1 based on steady state kinetic measurements. We found that LLC-PK1 cells, a model of the porcine kidney proximal tubular epithelium, robustly express Acy1. For the first time, we demonstrate uptake and utilization of N-acteylleucine and -methionine in replacement of the free amino acid, respectively, in cultured epithelial cells. Our data are consistent with a specific role of kidney Acy1 in the salvage of amino acids originating from systemic degradation of N-acetylated proteins.
机译:哺乳动物氨酰酶-1(Acy1)参与细胞内蛋白质分解代谢过程中N-乙酰化氨基酸的分解。 Acy 1在肾小管上皮中最丰富地表达。最近,在尿中几种N-乙酰氨基酸排泄增加的儿童中发现了Acy1缺乏症。在这里,我们报告基于稳态动力学测量的人和猪Acy1的详细N-乙酰氨基酸特异性概况。我们发现LLC-PK1细胞,猪肾近端肾小管上皮的模型,强烈表达Acy1。首次,我们证明了在培养的上皮细胞中N-乙酰基亮氨酸和-蛋氨酸的摄取和利用分别代替了游离氨基酸。我们的数据与肾脏Acy1在挽救源自N-乙酰化蛋白质的系统降解氨基酸的特定作用中是一致的。

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