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Open AccessJournal ArticleDOI

Quality control of mitochondria: protection against neurodegeneration and ageing

Takashi Tatsuta, +1 more
- 23 Jan 2008 - 
- Vol. 27, Iss: 2, pp 306-314
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TLDR
Current knowledge on surveillance strategies that limit mitochondrial damage and ensure cellular integrity and their role in human disease are summarized.
Abstract
Dysfunction of mitochondria has severe cellular consequences and is linked to ageing and neurodegeneration in human. Several surveillance strategies have evolved that limit mitochondrial damage and ensure cellular integrity. Intraorganellar proteases conduct protein quality control and exert regulatory functions, membrane fusion and fission allow mitochondrial content mixing within a cell, and the autophagic degradation of severely damaged mitochondria protects against apoptosis. Here, we will summarize the current knowledge on these surveillance strategies and their role in human disease.

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Citations
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Journal ArticleDOI

The axonal transport of mitochondria

TL;DR: Why mitochondria move and how they move is reviewed, focusing particularly on recent studies of transport regulation, which implicate control of motor activity by specific cell-signaling pathways, regulation of motor access to transport tracks and static microtubule–mitochondrion linkers.
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Essential role for Nix in autophagic maturation of erythroid cells

TL;DR: It is suggested that Nix-dependent loss of ΔΨm is important for targeting the mitochondria into autophagosomes for clearance during erythroid maturation, and interference with this function impairs erythyroidmaturation and results in anaemia.
Journal ArticleDOI

Mitochondria-Anchored Receptor Atg32 Mediates Degradation of Mitochondria via Selective Autophagy

TL;DR: It is demonstrated that, in post-log phase cells under respiratory conditions, a substantial fraction of mitochondria are exclusively sequestered as cargoes and transported to the vacuole, a lytic compartment in yeast, in an autophagy-dependent manner and suggested that Atg32, a mitochondria-anchored protein essential for mitophagy that is induced during respiratory growth, interacts with Atg8 and Atg11, autophatic proteins critical for recognition of cargo receptors.
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Mitochondrial import efficiency of ATFS-1 regulates mitochondrial UPR activation.

TL;DR: The mechanism by which ATFS-1 (activating transcription factor associated with stress–1) senses mitochondrial stress and communicates with the nucleus during the mitochondrial unfolded protein response (UPRmt) in Caenorhabditis elegans is examined and it is found that the key point of regulation is the mitochondrial import efficiency of ATfs-1.
Journal ArticleDOI

Mitochondrial transport in neurons: impact on synaptic homeostasis and neurodegeneration

TL;DR: Research into the mechanisms that regulate mitochondrial transport is an important emerging frontier into the pathogenesis of several major neurological disorders.
References
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Journal ArticleDOI

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

TL;DR: Treatments targeting basic mitochondrial processes, such as energy metabolism or free-radical generation, or specific interactions of disease-related proteins with mitochondria hold great promise in ageing-related neurodegenerative diseases.
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Mitochondrial Membrane Permeabilization in Cell Death

TL;DR: Once MMP has been induced, it causes the release of catabolic hydrolases and activators of such enzymes (including those of caspases) from mitochondria, meaning that mitochondria coordinate the late stage of cellular demise.
Journal ArticleDOI

Self-eating and self-killing: crosstalk between autophagy and apoptosis

TL;DR: The functional relationship between apoptosis and autophagy is complex in the sense that, under certain circumstances,autophagy constitutes a stress adaptation that avoids cell death (and suppresses apoptosis), whereas in other cellular settings, it constitutes an alternative cell-death pathway.
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Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development

TL;DR: It is concluded that Mfn1 and Mfn2 have both redundant and distinct functions and act in three separate molecular complexes to promote mitochondrial fusion, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population.
Journal ArticleDOI

Function and regulation of cullin-RING ubiquitin ligases.

TL;DR: This review focuses on the composition, regulation and function of cullin–RING ligases, and describes how these enzymes can be characterized by a set of general principles.
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