2015
DOI: 10.18632/aging.100812
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Abstract: The inactivation of the LRPPRC gene, which has previously been associated with the neurodegenerative French Canadian Leigh Syndrome, results in a decrease in the production of mitochondria-encoded subunits of complex IV, thereby causing a reduction in complex IV activity. Previously we have shown that reducing complex IV activity triggers a compensatory and conserved mitochondrial hyperfusion response. We now demonstrate that LRPPRC knock-down in mammalian cells leads to an imbalance between mitochondria-encod… Show more

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Cited by 20 publications
(13 citation statements)
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References 32 publications
(56 reference statements)
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“…Remarkably, the levels of HSP60 were only mildly increased in CLPP‐deficient cells, in agreement with our in vivo data on DARS2‐deficient hearts (Figs C and EV3A and C). Similarly, upregulation of mtHSP70, but not HSP60 levels, is observed in mammalian cells depleted of LRPPRC, a key posttranscriptional regulator of mtDNA expression . The knockdown of LRPPRC and its C. elegans homolog mma‐1 induces mito‐nuclear imbalance and UPR mt in both model organisms .…”
Section: Resultsmentioning
confidence: 96%
“…Remarkably, the levels of HSP60 were only mildly increased in CLPP‐deficient cells, in agreement with our in vivo data on DARS2‐deficient hearts (Figs C and EV3A and C). Similarly, upregulation of mtHSP70, but not HSP60 levels, is observed in mammalian cells depleted of LRPPRC, a key posttranscriptional regulator of mtDNA expression . The knockdown of LRPPRC and its C. elegans homolog mma‐1 induces mito‐nuclear imbalance and UPR mt in both model organisms .…”
Section: Resultsmentioning
confidence: 96%
“…As a positive control, we used animals carrying a lf mutation of the gene spg-7 AFG3L2 (spg-7(ad2249)), which encodes a mitochondrial metalloprotease required for mitochondrial function [41]. The zcIs13 transgene shows very low baseline expression in wild-type animals and is widely used to monitor UPR mt in C. elegans [7,[9][10][11][12][13][14][42][43][44]. In the case of fzo-1(tm1133) animals, for example, its expression is induced more than 15-fold (S1C Fig).…”
Section: Resultsmentioning
confidence: 99%
“…To detect GFP and Tubulin, we used primary anti-GFP (1:1000, Roche 11814460001) and primary anti-α-Tubulin (1:5000, Abcam ab7291) antibodies and secondary horseradish peroxidase-conjugated goat anti-mouse antibodies (BioRad #1706516). To detect endogenous HSP-6, we used anti-HSP-6 (1:10,000) as described previously [42] and secondary horseradish peroxidase-conjugated goat anti-rabbit antibodies (BioRad #1706515). Blots were developed using ECL (Amersham) or ECL Prime (Amersham) according to manufacturer's protocol and images were quantified using the ChemiDoc XRS+ System (BioRad).…”
Section: Western Blot Analysismentioning
confidence: 99%
“…We speculate the PGC-1α/LRPPRC complex can reduce the damage of mitochondrial oxidative stress by inhibiting the ubiquitin/proteasome system. Knocking out LRPPRC in mammalian cells leads to the imbalance of the complex IV subunit of the mitochondrial and nuclear co-coding complex, which triggers the mtUPR 60 . Thus, we believe that PGC-1α/LRPPRC largely regulates mtUPR.…”
Section: Discussionmentioning
confidence: 99%