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ATFS-1 counteracts mitochondrial DNA damage by promoting repair over transcription
The ability to balance conflicting functional demands is critical for ensuring organismal survival. The transcription and repair of the mitochondrial genome (mtDNA) requires separate enzymatic activities that ...
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LONP-1 and ATFS-1 sustain deleterious heteroplasmy by promoting mtDNA replication in dysfunctional mitochondria
The accumulation of deleterious mitochondrial DNA (∆mtDNA) causes inherited mitochondrial diseases and ageing-associated decline in mitochondrial functions such as oxidative phosphorylation. Following mitochon...
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Article
Open AccessGenome-wide detection of CRISPR editing in vivo using GUIDE-tag
Analysis of off-target editing is an important aspect of the development of safe nuclease-based genome editing therapeutics. in vivo assessment of nuclease off-target activity has primarily been indirect (base...
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Article
Open AccessUPRmt scales mitochondrial network expansion with protein synthesis via mitochondrial import in Caenorhabditis elegans
As organisms develop, individual cells generate mitochondria to fulfill physiological requirements. However, it remains unknown how mitochondrial network expansion is scaled to cell growth. The mitochondrial u...
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Open AccessUPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication
The mitochondrial network is not only required for the production of energy, essential cofactors and amino acids, but also serves as a signaling hub for innate immune and apoptotic pathways. Multiple mechanism...
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The mitochondrial UPR: mechanisms, physiological functions and implications in ageing
The mitochondrial unfolded protein response (UPRmt) is a conserved transcriptional response activated by multiple forms of mitochondrial dysfunction and regula...
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The mitokine quest(ion)
Cells and organisms adapt to mitochondrial dysfunction by activating the mitochondrial unfolded protein response (UPRmt), which is regulated by mitochondrial-to-nuclear communication; and UPRmt activation can als...
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Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response
In the context of mitochondrial genome heteroplasmy that causes defective oxidative phosphorylation in C. elegans, the ATFS-1-mediated mitochondrial unfolded protein response maintains the deleterious mitochondri...
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Surviving import failure
Two studies reveal that dysfunction in organelles called mitochondria causes the toxic accumulation of mitochondrial proteins in the cell's cytosolic fluid, and identify ways in which damage is mitigated. See Let...
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Alan Hall 1952–2015
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Open AccessMitophagy and the mitochondrial unfolded protein response in neurodegeneration and bacterial infection
Mitochondria are highly dynamic and structurally complex organelles that provide multiple essential metabolic functions. Mitochondrial dysfunction is associated with neurodegenerative conditions such as Parkin...
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Mitochondrial UPR-regulated innate immunity provides resistance to pathogen infection
A link between an intracellular stress response, bacterial infection and triggering of the innate immune response is shown in Caenorhabditis elegans; exposure to the pathogen Pseudomonas aeruginosa caused activat...
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Autophagy machinery mediates macroendocytic processing and entotic cell death by targeting single membranes
Autophagy normally involves the formation of double-membrane autophagosomes that mediate bulk cytoplasmic and organelle degradation. Here we report the modification of single-membrane vacuoles in cells by auto...
