scholarly journals Multilevel heterogeneity of mitochondrial respiratory chain deficiency

2018 ◽  
Vol 246 (3) ◽  
pp. 261-265 ◽  
Author(s):  
Amy E Vincent ◽  
Martin Picard
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Respiratory Chain Deficiency ◽  
Mitochondrial Respiratory

Antenatal manifestations of mitochondrial respiratory chain deficiency

2003 ◽  
Vol 143 (2) ◽  
pp. 208-212 ◽  
Author(s):  
Jürgen-Christoph von Kleist-Retzow ◽  
Valérie Cormier-Daire ◽  
Géraldine Viot ◽  
Alice Goldenberg ◽  
Becky Mardach ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Respiratory Chain Deficiency ◽  
Mitochondrial Respiratory

Mitochondrial respiratory chain deficiency leads to overexpression of antioxidant enzymes

FEBS Letters ◽  
1997 ◽  
Vol 418 (3) ◽  
pp. 247-250 ◽  
Author(s):  
Liliana Brambilla ◽  
Gaetano Cairo ◽  
Piero Sestili ◽  
Valery O'Donnel ◽  
Angelo Azzi ◽  
...  
Keyword(s):  
Antioxidant Enzymes ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Respiratory Chain Deficiency ◽  
Mitochondrial Respiratory

scholarly journals Mitochondrial respiratory chain deficiency inhibits lysosomal hydrolysis

Autophagy ◽  
2019 ◽  
Vol 15 (9) ◽  
pp. 1572-1591 ◽  
Author(s):  
Lorena Fernandez-Mosquera ◽  
King Faisal Yambire ◽  
Renata Couto ◽  
Leonardo Pereyra ◽  
Kamil Pabis ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Respiratory Chain Deficiency ◽  
Mitochondrial Respiratory

Defective valyl-tRNA synthetase hampers the mitochondrial respiratory chain in Neurospora crassa

2012 ◽  
Vol 448 (3) ◽  
pp. 297-306 ◽  
Author(s):  
Margarida Duarte ◽  
Arnaldo Videira
Keyword(s):  
Protein Synthesis ◽  
Neurospora Crassa ◽  
Mutant Strain ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Trna Synthetase ◽  
Wild Type ◽  
Respiratory Chain Deficiency ◽  
Cytosolic Protein ◽  
Mitochondrial Respiratory

Respiratory chain deficiency can result from alterations in mitochondrial and/or cytosolic protein synthesis due to the dual genetic origin of mitochondrial oxidative phosphorylation. In the present paper we report a point mutation (D750G) in the bifunctional VARS (valyl-tRNA synthetase) of the fungus Neurospora crassa, associated with a temperature-sensitive phenotype. Analysis of the mutant strain revealed decreased steady-state levels of VARS and a clear reduction in the rate of mitochondrial protein synthesis. We observed a robust induction of the mitochondrial alternative oxidase with a concomitant decrease in the canonical respiratory pathway, namely in cytochrome b and aa3 content. Furthermore, the mutant strain accumulates the peripheral arm of complex I and depicts decreased levels of complexes III and IV, consistent with severe impairment of the mitochondrial respiratory chain. The phenotypic alterations of the mutant strain are observed at the permissive growth temperature and exacerbated upon increase of the temperature. Surprisingly, glucose-6-phosphate dehydrogenase activities were similar in the wild-type and mutant strains, whereas mitochondrial activities for succinate dehydrogenase and alternative NADH dehydrogenases were increased in the mutant strain, suggesting that the VARSD−G mutation does not affect overall cytosolic protein synthesis. Expression of the wild-type vars gene rescues all of the mutant phenotypes, indicating that the VARSD−G mutation is a loss-of-function mutation that results in a combined respiratory chain deficiency.

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