The cytoplasmic peptide:N-glycanase (NGLY1) - Structure, expression and cellular functions.
The cytoplasmic peptide:N-glycanase (NGLY1) - Structure, expression and cellular functions.
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DOI：
10.1016/j.gene.2015.11.021
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发表时间：
2016-02-10
期刊：
影响因子：
3.5
通讯作者：
Fujihira H
中科院分区：
文献类型：
--
作者：
Suzuki T;Huang C;Fujihira H
NGLY 1/Ngly 1是一种胞质肽：N-聚糖酶，即在哺乳动物中作用于N-糖蛋白的去N-糖基化酶，产生游离的、未缀合的N-聚糖和去糖基化肽，其中N-糖基化天冬酰胺残基转化为谷氨酸盐。已知这种酶参与内质网（ER）中新合成糖蛋白的质量控制系统。在该系统中，错误折叠的（糖）蛋白被逆转运到胞质溶胶中，其中26 S蛋白酶体在降解蛋白质中发挥核心作用：这一过程被称为ER相关降解或简称ERAD。PNGase介导的去糖基化被认为促进一些错误折叠的糖蛋白的有效降解。最近发现了携带NGLY 1基因突变（NGLY 1缺陷）的人类患者，清楚地表明了这种酶的功能重要性。本文综述了NGLY 1及其基因产物在哺乳动物细胞中的研究进展。
NGLY1/Ngly1 is a cytosolic peptide:N-glycanase, i.e. de-N-glycosylating enzyme acting on N-glycoproteins in mammals, generating free, unconjugated N-glycans and deglycosylated peptides in which the N-glycosylated asparagine residues are converted to aspartates. This enzyme is known to be involved in the quality control system for the newly synthesized glycoproteins in the endoplasmic reticulum (ER). In this system, misfolded (glyco)proteins are retrotranslocated to the cytosol, where the 26S proteasomes play a central role in degrading the proteins: a process referred to as ER-associated degradation or ERAD in short. PNGase-mediated deglycosylation is believed to facilitate the efficient degradation of some misfolded glycoproteins. Human patients harboring mutations of NGLY1 gene (NGLY1-deficiency) have recently been discovered, clearly indicating the functional importance of this enzyme. This review summarizes the current state of our knowledge on NGLY1 and its gene product in mammalian cells.