Abstract
Mitochondria play a crucial role in cell life and death by regulating bioenergetic and biosynthetic pathways. They are able to adapt rapidly to different microenvironmental stressors by accommodating the metabolic and biosynthetic needs of the cell. Mounting evidence places mitochondrial dysfunction at the core of several diseases, notably in the context of pathologies of the cardiovascular and central nervous system. In addition, mutations in some mitochondrial proteins are bona fide cancer drivers. Better understanding of the functions of these multifaceted organelles and their components may finetune our knowledge on the molecular bases of certain diseases and suggest new therapeutic avenues.
Highlights
Mitochondria are semi-autonomous organelles with a double membrane system, namely the inner and the outer mitochondrial membrane that delimit the intermembrane space
Mitochondria contain their own genome, the mitochondrial DNA, a circular double-stranded DNA molecule of 16,569 bp in humans, which encodes only 13 mitochondrial proteins belonging to the electron transport chain (ETC), 22 transfer RNAs and 2 ribosomal RNAs needed to carry out the mitochondrial protein synthesis
Mitochondrial dysfunction is implicated in several pathological conditions, ranging from neurodegenerative and cardiovascular diseases, to aging, cancer and inflammation
Summary
Mitochondria are semi-autonomous organelles with a double membrane system, namely the inner and the outer mitochondrial membrane that delimit the intermembrane space. The first primary mitochondrial disease was described in 1962 [14] and involved a 35-year-old woman displaying excessive perspiration, polyphagia, polydipsia without polyuria, asthenia and decreased body weight, symptoms that started when she was seven years old Her basal metabolic rate was +172%, and she presented with creatinuria, myopathy and pathological cardiomyogram. Environmental stress can induce mtDNA alterations leading to mitochondrial dysfunction during aging, inflammatory response, etc. Immune cells under a hyper-inflammatory state metabolically adapt to this stress condition by favoring aerobic glycolysis over OXPHOS for energy production This metabolic rewiring allows macrophages to become more phagocytic and favors anabolic reactions for the synthesis and secretion of cytokines and chemokines in a vicious cycle ([30] and references therein). Genetic manipulations in C. elegans that increase mitophagy extend the organismal lifespan [45], strengthening the connection between altered mitophagy and aging
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