LONP1 is the major protease responsible for the recognition and removal of unfolded proteins in the mitochondrial matrix and thereby protects the cell from the accumulation of aggregated proteins in the mitochondrion. [10]

这个基因编码的线粒体基质蛋白质属于 ATP依赖的Lon蛋白酶家族，类似于 大肠杆菌 基因编码的特异性调控目标蛋白降解的蛋白。 这种蛋白质结合在线粒体基因组启动子的轻链和重链的特异性序列上，参与DNA复制和转录的调控 [8]。 ^ 與LONP1相關的疾病；在維基數據上查看/編輯參考. ^ Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. ^ Mouse PubMed Reference:.

2024年12月25日 · LONP1 (Lon Peptidase 1, Mitochondrial) is a Protein Coding gene. Diseases associated with LONP1 include Codas Syndrome and Pyruvate Dehydrogenase E1-Alpha Deficiency. Among its related pathways are Cellular responses to …

2023年5月24日 · 线粒体离子肽酶1 (lon peptidase 1,mitochondrial,LONP1)是由核DNA编码的ATP依赖的蛋白质，位于线粒体基质中，在调节线粒体基因的表达、维持线粒体稳定等方面具有重要作用。 线粒体稳态（线粒体稳态是维持细胞正常生理活动的关键，该稳态主要通过对线粒体相关的溶酶体，线粒体自噬，蛋白酶等物质的调节实现）的破坏是正常细胞发生致瘤性转化的普遍特性。 线粒体离子肽酶1 (lon peptidase 1,mitochondrial,LONP1)是控制线粒体质量的主要蛋白之 …

2021年1月11日 · We show that LONP1, an AAA+ protease of the mitochondrial matrix, works with the mtHSP70 chaperone system to promote mitochondrial protein folding. Inhibition of LONP1 results in aggregation of...

Initially discovered as a protease responsible for degradation of misfolded or damaged proteins, the mitochondrial Lon protease (Lonp1) turned out to be a multifaceted enzyme, that displays at least three different functions (proteolysis, chaperone activity, …

2021年8月16日 · Human ATP-dependent Lon protease (LONP1) forms homohexameric, ring-shaped complexes. Depletion of LONP1 causes aggregation of a broad range of proteins in the mitochondrial matrix and...

Lon peptidase 1 (LONP1) plays a vital role in the proliferation and metastasis of pancreatic cancer, which provides a potential therapeutic target for the treatment of pancreatic cancer. Identify LonP1 as a key effector of mitochondrial reprogramming.

2023年5月24日 · 该研究发现了依赖蛋白酶lonp1的线粒体蛋白水解重排调控白色脂肪细胞的命运转变；揭示了高度调控的线粒体蛋白酶是细胞的关键感应纽带，将细胞外环境刺激、线粒体呼吸链复合物/代谢重排与白色脂肪产热重塑过程中的细胞命运重编程有机联系起来。

2025年2月19日 · Lon protease 1 (LONP1) is an ATP-dependent protease located in the mitochondrial matrix and plays a crucial role in regulating mitochondrial proteostasis, metabolism, and...