Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction
Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction
复制标题
糖衣自噬：探索抑制 NGLY1（N-聚糖酶 1）与自噬诱导之间的联系
DOI：
10.1080/27694127.2023.2166324
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Kramer H
中科院分区：
文献类型：
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作者：
Kramer H
胞质酶NGLY1 （n -聚糖酶1）是糖蛋白分解代谢的中心介质。在蛋白酶体降解错误折叠的蛋白质之前，该酶从修饰的底物天冬酰胺残基中清除与蛋白连接的聚糖，在er相关的降解/ERAD途径中起着关键且保守的作用。在临床背景下，NGLY1疾病代表了一种罕见的先天性去糖基化疾病，其中engly1基因突变导致酶功能丧失。NGLY1障碍患者表现出广泛而多样的症状，包括中度至重度发育迟缓、癫痫发作、复杂的运动障碍以及白斑。我们最近的研究结果强调了NGLY1抑制与巨噬/自噬诱导之间的因果关系。
The cytosolic enzyme NGLY1 (N-glycanase 1) is a central mediator of glycoprotein catabolism. The enzyme acts to cleaveN-linked glycans from modified substrate asparagine residues prior to degradation of misfolded proteins by the proteasome, playing a key and well-conserved role in the ER-associated degradation/ERAD pathway. In a clinical context, NGLY1 disorder represents a rare congenital disorder of deglycosylation where mutations in theNGLY1gene result in the loss of enzyme function. Patients with NGLY1 disorder present with a broad and varied array of symptoms, which can include moderate to profound levels of developmental delay, seizures, and complex movement disorders, as well as alacrima. Our recent results highlight a causal link between NGLY1 inhibition and macroautophagy/autophagy induction.