Starvation-induced endosomal recruitment of MTM1 impairs PI(3)P-dependent contact formation between tubular ER membranes and early endosomes, resulting in the conversion of ER tubules into sheets, the inhibition of mitochondrial fission, and sustained oxidative metabolism.

2022年12月16日 · We show that endosomal signaling lipid turnover by MTM1, a phosphatidylinositol 3-phosphate [PI(3)P] 3-phosphatase mutated in X-linked centronuclear myopathy in humans, controls mitochondrial morphology and function by reshaping the endoplasmic reticulum (ER).

2022年12月26日 · 这种疾病的遗传缺陷影响到脂质磷酸酶mtm1。 这种酶控制着内体（endosome）上一种脂质信号分子的周转，内体是细胞中参与营养受体分选的囊泡状结构。

2024年11月1日 · Hydrolysis of PI(3)P on early endosomes by MTM1 during starvation causes a reduction in MCSs between the ER and early endosomes, thereby interfering with tubular ER hitchhiking, ultimately resulting in the transformation of tubules into sheet-like ER structures [19].

2022年12月15日 · In nutrient deprivation, MTM1 is recruited to early endosomes, where it hydrolyzes PI(3)P. This releases the endosome-ER connection, resulting in ER sheet morphology, decreased mitochondrial fission, and reduced lipid droplet biogenesis.

We show that endosomal signaling lipid turnover by MTM1, a phosphatidylinositol 3-phosphate [PI(3)P] 3-phosphatase mutated in X-linked centronuclear myopathy in humans, controls mitochondrial morphology and function by reshaping the endoplasmic reticulum (ER).

2013年4月15日 · In this study, we identified myotubularin (MTM1), a phosphoinositide phosphatase mutated in X-linked centronuclear myopathy (XLCNM, or myotubular myopathy), as a key regulator of phosphatidylinositol 3-monophosphate (PtdIns3 P) levels at the SR.

We show that endosomal signaling lipid turnover by MTM1, a phosphatidylinositol 3-phosphate [PI (3)P] 3-phosphatase mutated in X-linked centronuclear myopathy in humans, controls mitochondrial morphology and function by reshaping the endoplasmic reticulum (ER).

2022年12月15日 · 饥饿诱导的 mtm1 内体募集损害了管状 er 膜和早期内体之间的 pi(3)p 依赖性接触形成，导致 er 小管转化为片层，抑制线粒体裂变，并持续氧化代谢。 我们的结果揭示了早期内体脂质信号在控制 ER 形状方面的重要作用，因此，

2022年12月16日 · We show that endosomal signaling lipid turnover by MTM1, a phosphatidylinositol 3-phosphate [PI (3)P] 3-phosphatase mutated in X-linked centronuclear myopathy in humans, controls mitochondrial...