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The mitochondrial function maintenance pathway, frequently termed mitochondrial quality control (MQC), is an integrated cellular system responsible for preserving the structural and functional integrity of the mitochondrial network (Pickering et al., 2018, Nature Reviews Molecular Cell Biology). This pathway encompasses three primary pillars: mitochondrial biogenesis (the creation of new mitochondria), mitochondrial dynamics (the processes of fission and fusion), and mitophagy (the selective autophagic degradation of damaged mitochondria) (Youle & Narendra, 2011, Nature). Central regulators such as PGC-1α, AMPK, and the PINK1/Parkin axis coordinate these processes to maintain energy homeostasis, regulate calcium levels, and mitigate oxidative stress (Lin et al., 2005, Cell Metabolism). Dysregulation of this pathway is a critical factor in the etiology of neurodegenerative diseases, such as Parkinson's and Alzheimer's, as well as metabolic disorders like type 2 diabetes and cardiovascular diseases (Swerdlow, 2018, Journal of Alzheimer's Disease). Therapeutic approaches targeting this pathway involve the use of small molecules to activate metabolic sensors or stabilize mitochondrial membranes, aiming to restore cellular vitality and prevent disease progression (Murphy & Hartley, 2018, Nature Reviews Drug Discovery).
Activation of mitochondrial biogenesis via AMPK/SIRT1/PGC-1α signaling, stabilization of mitochondrial inner membrane lipids (cardiolipin), and induction of mitophagy to clear dysfunctional organelles.
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