Optic atrophy 1 (OPA1) is a mitochondrial dynamin-like GTPase that controls mitochondrial morphogenesis, fusion, and energetics. Here, we report that human and mouse cells lacking OPA1 are markedly resistant to ferroptosis.

2025年3月30日 · OPA1 (OPA1 Mitochondrial Dynamin Like GTPase) is a Protein Coding gene. Diseases associated with OPA1 include Behr Syndrome and Mitochondrial Dna Depletion Syndrome 14. Among its related pathways are Programmed Cell Death and Autodegradation of the E3 ubiquitin ligase COP1.

opa1. 在塑造imm中，opa1起着另一个主要作用：作为imm融合的中介。opa1基因缺失导致线粒体网络的断裂，而过表达opa1可以通过mfn1促进延伸，这表明opa1与mfn1的ims环相互作用或间接影响mfn1融合活性。 imm融合的机制以及该反应所必需的病理结构域尚不清楚。

2023年10月15日 · 在众多线粒体动态变化调节因子中，机械化学GTP酶OPA1（optic atrophy 1） 是至关重要的一个（图1），OPA1在体内以两种形式存在：位于线粒体内膜上的跨膜长形式l-OPA1与线粒体内膜蛋白水解产生的短的可溶性形式s-OPA1。 OPA1催化线粒体内膜的融合并调节线粒体嵴重塑过程，对于骨骼肌、血管、神经元等分化细胞的形成和功能产生巨大影响【1,2】。 图1. 线粒体融合图解、s-OPA1序列图及其GDP-AlFx模型. 显性视神经萎缩是导致小朋友失明 …

在本篇文章中，我们主要从结构、功能、调节和相关疾病4个方面详细介绍了线粒体蛋白酶OMA1。 1. OMA1结构. 2. OMA1功能. 3. OPA1-OMA1调控机制. 4. OMA1与疾病. 1. OMA1结构. Figure 2. The location of MOA1 gene. 人蛋白金属内切酶OMA1由524个氨基酸组成，其中包含了位于1-13的氨基酸的信号肽，分子量大小为60.1 kDa，成熟OMA1蛋白理论上的pI为8.44 [3]。 该蛋白之所以被称为OMA1，是因为它与 m-AAA蛋白酶 具有重叠活性 [4]。 OMA1的人类直系同源基因 …

2023年8月23日 · Our findings provide a structural framework for understanding how human OPA1 shapes mitochondrial morphology and show us how human disease mutations compromise OPA1 functions. Eukaryotic cells...

2023年10月23日 · Optic atrophy 1 (OPA1) is a mitochondrial fusion protein with a role in cristae shaping. Disruption of OPA1 causes mitochondrial dysfunction. However, the role of OPA1 in liver function...

The OPA1 gene provides instructions for making a protein that is found in cells and tissues throughout the body. The OPA1 protein is active in the inner membrane of cell structures called mitochondria, which are the energy-producing centers in cells.

2019年9月24日 · Optic atrophy 1 (OPA1) is one of the factors that control mitochondrial fusion, mitochondrial DNA (mtDNA) maintenance, bioenergetics, and cristae integrity. In this...

In OPA1 variants, this results in mitochondrial structural fragmentation, impaired adenosine triphosphate generation, and increased susceptibility to cell death. Therapeutically, this can be addressed by stimulating the expression of the remaining normal allele to protect or rescue mitochondrial function.