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article

The PERK Arm of the Unfolded Protein Response Regulates Mitochondrial Morphology during Acute Endoplasmic Reticulum Stress

Justine Lebeau, Jaclyn M. Saunders, Vivian W.R. Moraes, Aparajita Madhavan, more

Cell Reports > 2018 > 22 > 11 > 2827-2836

Endoplasmic reticulum (ER) stress is transmitted to mitochondria and is associated with pathologic mitochondrial dysfunction in diverse diseases. The PERK arm of the unfolded protein response (UPR) protects mitochondria during ER stress through the transcriptional and translational remodeling of mitochondrial molecular quality control pathways. Here, we show that ER stress also induces dynamic remodeling...

article

Mitochondrial response to the BCKDK-deficiency: Some clues to understand the positive dietary response in this form of autism

A. Oyarzabal, I. Bravo-Alonso, M. Sánchez-Aragó, M.T. Rejas, more

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease > 2016 > 1862 > 4 > 592-600

Mutations on the mitochondrial-expressed Branched Chain α-Keto acid Dehydrogenase Kinase (BCKDK) gene have been recently associated with a novel dietary-treatable form of autism. But, being a mitochondrial metabolism disease, little is known about the impact on mitochondrial performance. Here, we analyze the mitochondrial response to the BCKDK-deficiency in patient's primary fibroblasts by measuring...

article

Human mitochondrial Fis1 links to cell cycle regulators at G2/M transition

Seungmin Lee, Yong-Yea Park, Song-Hee Kim, Oanh T. Kim Nguyen, more

Cellular and Molecular Life Sciences > 2014 > 71 > 4 > 711-725

We have previously shown that prolonged mitochondrial elongation triggers cellular senescence. Here, we report that enforced mitochondrial elongation by hFis1 depletion caused a severe defect in cell cycle progression through G2/M phase (~3-fold reduction in mitotic index; p < 0.01). Reintroduction of Myc-hFis1 to these cells induced mitochondrial fragmentation and restored the cell cycle, indicating...

article

Parkinson’s disease‐associated DJ‐1 mutations impair mitochondrial dynamics and cause mitochondrial dysfunction

Xinglong Wang, Timothy G. Petrie, Yingchao Liu, Jun Liu, more

Journal of Neurochemistry > 121 > 5 > 830 - 839

J. Neurochem. (2012) 121, 830–839. AbstractMitochondrial dysfunction represents a critical event during the pathogenesis of Parkinson’s disease (PD) and expanding evidences demonstrate that an altered balance in mitochondrial fission/fusion is likely an important mechanism leading to mitochondrial and neuronal dysfunction/degeneration. In this study, we investigated whether DJ‐1 is involved in the...

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Keywords

MITOCHONDRIAL ELONGATION (4)
AUTISM (1)
BCKDK-DEFICIENCY (1)
BIOENERGETICS PROFILE (1)
DJ‐1 (1)
DRP1 (1)
EIF2Α PHOSPHORYLATION (1)
ENDOPLASMIC RETICULUM STRESS (1)
FOXM1 (1)
G2/M PROGRESSION (1)
HFIS1 (1)
MITOCHONDRIAL DISEASE (1)
MITOCHONDRIAL FRAGMENTATION (1)
MITOCHONDRIAL FUSION (1)
MITOCHONDRIAL PROTEOSTASIS (1)
PARKINSON DISEASE (1)
PERK (1)
PLK1 (1)
STRESS-INDUCED MITOCHONDRIAL HYPERFUSION (1)
UNFOLDED PROTEIN RESPONSE (1)
UNRESTRAINED BRANCHED-CHAIN AMINO ACIDS CATABOLISM (1)
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The PERK Arm of the Unfolded Protein Response Regulates Mitochondrial Morphology during Acute Endoplasmic Reticulum Stress

Mitochondrial response to the BCKDK-deficiency: Some clues to understand the positive dietary response in this form of autism

Human mitochondrial Fis1 links to cell cycle regulators at G2/M transition

Parkinson’s disease‐associated DJ‐1 mutations impair mitochondrial dynamics and cause mitochondrial dysfunction

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