Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease.

Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease.
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DOI:
10.1016/j.freeradbiomed.2022.05.005
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发表时间:
2022-06
影响因子:
7.4
通讯作者:
Paul, Bindu D.
Paul, Bindu D.
中科院分区:
医学1区
文献类型:
--
作者:
Paul, Bindu D.

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半必需氨基酸半胱氨酸在必需的细胞过程以及信号级联的调节中起重要作用。半胱氨酸既可从饮食中获得,也可通过转硫途径内源性产生。半胱氨酸进一步用于蛋白质合成和各种含硫分子的生物合成。半胱氨酸催化剂的产物之一硫化氢(H2S)是一种气体信号分子,其调节多种细胞过程。半胱氨酸代谢在几种神经退行性疾病和衰老过程中失调。这篇小综述主要关注亨廷顿病中异常的半胱氨酸和H2S代谢,亨廷顿病是一种神经退行性疾病,由亨廷顿基因中多聚谷氨酰胺编码重复序列的扩增引起,导致运动和认知缺陷。
The semi-essential amino acid, cysteine, plays important roles in both essential cellular processes as well as in modulation of signaling cascades. Cysteine is obtained both from the diet as well as generated endogenously via the transsulfuration pathway. Cysteine is further utilized in protein synthesis and biosynthesis of various sulfur containing molecules. One of the products of cysteine catabolism, hydrogen sulfide (H2S), is a gaseous signaling molecule, which regulates a multitude of cellular processes. Cysteine metabolism is dysregulated in several neurodegenerative diseases and during aging. This minireview focuses on aberrant cysteine and H2S metabolism in Huntington’s disease, a neurodegenerative disease caused by expansion of polyglutamine encoding repeats in the gene huntingtin, which leads to motor and cognitive deficits.
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