Mitochondria produce the bulk of cellular energy and work as decisional "hubs" for cellular responses by integrating different input signals. The determinant in the physiopathology of mammals, they attract major attention, nowadays, for their contribution to brain degeneration. How they can withstand or succumb to insults leading to neuronal death is an object of great attention increasing the need for a better understanding of the interplay between inner and outer mitochondrial pathways residing in the cytosol. Of the latter, those dictating protein metabolism and therefore influencing the quality function and control of the organelle are of our most immediate interest and here we describe the Transglutaminase type 2 (TG2) contribution to mitochondrial function, dysfunction and neurodegeneration. Besides reviewing the latest evidences we share also the novel ones on the IF1 pathway depicting a molecular conduit governing mitochondrial turnover and homeostasis relevant to envisaging preventive and therapeutic strategies to respectively predict and counteract deficiencies associated with deregulated mitochondrial function in neuropathology.

中文翻译：

---

2 型转谷氨酰胺酶、线粒体和亨廷顿病：管理三足动物

线粒体产生大量细胞能量，并通过整合不同的输入信号作为细胞反应的决策“中心”。它们是哺乳动物病理生理学的决定因素，如今由于它们对脑退化的贡献而引起了极大的关注。它们如何承受或屈服于导致神经元死亡的侮辱是一个备受关注的对象，这增加了对驻留在细胞质中的内部和外部线粒体通路之间相互作用的更好理解的需求。在后者中，决定蛋白质代谢并因此影响细胞器质量功能和控制的那些是我们最直接的兴趣，在这里我们描述转谷氨酰胺酶 2 (TG2) 对线粒体功能、功能障碍和神经变性的贡献。