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- W3183806972 endingPage "833" @default.
- W3183806972 startingPage "833" @default.
- W3183806972 abstract "Mounting evidence shows a link between mitochondrial dysfunction and neurodegenerative disorders, including Alzheimer Disease. Increased oxidative stress, defective mitodynamics, and impaired oxidative phosphorylation leading to decreased ATP production, can determine synaptic dysfunction, apoptosis, and neurodegeneration. Furthermore, mitochondrial proteostasis and the protease-mediated quality control system, carrying out degradation of potentially toxic peptides and misfolded or damaged proteins inside mitochondria, are emerging as potential pathogenetic mechanisms. The enzyme pitrilysin metallopeptidase 1 (PITRM1) is a key player in these processes; it is responsible for degrading mitochondrial targeting sequences that are cleaved off from the imported precursor proteins and for digesting a mitochondrial fraction of amyloid beta (Aβ). In this review, we present current evidence obtained from patients with PITRM1 mutations, as well as the different cellular and animal models of PITRM1 deficiency, which points toward PITRM1 as a possible driving factor of several neurodegenerative conditions. Finally, we point out the prospect of new diagnostic and therapeutic approaches." @default.
- W3183806972 created "2021-08-02" @default.
- W3183806972 creator A5005070331 @default.
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- W3183806972 creator A5056933907 @default.
- W3183806972 creator A5070209892 @default.
- W3183806972 creator A5087383118 @default.
- W3183806972 creator A5087608233 @default.
- W3183806972 date "2021-07-17" @default.
- W3183806972 modified "2023-10-15" @default.
- W3183806972 title "Role of PITRM1 in Mitochondrial Dysfunction and Neurodegeneration" @default.
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