scholarly journals Infantile Progressive Hepatoencephalomyopathy with Combined OXPHOS Deficiency due to Mutations in the Mitochondrial Translation Elongation Factor Gene GFM1

2011 ◽  
pp. 113-122 ◽  
Author(s):  
S. Balasubramaniam ◽  
Y. S. Choy ◽  
A. Talib ◽  
M. D. Norsiah ◽  
L. P. van den Heuvel ◽  
...  
Keyword(s):  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation ◽  
Factor Gene

scholarly journals Chaperone Properties of Mammalian Mitochondrial Translation Elongation Factor Tu

2007 ◽  
Vol 282 (6) ◽  
pp. 4076-4084 ◽  
Author(s):  
Hiroaki Suzuki ◽  
Takuya Ueda ◽  
Hideki Taguchi ◽  
Nono Takeuchi
Keyword(s):  
Elongation Factor ◽  
Elongation Factor Tu ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation

scholarly journals Mutation in the mitochondrial translation elongation factor EFTs results in severe infantile liver failure

2012 ◽  
Vol 56 (1) ◽  
pp. 294-297 ◽  
Author(s):  
Vanessa Vedrenne ◽  
Louise Galmiche ◽  
Dominique Chretien ◽  
Pascale de Lonlay ◽  
Arnold Munnich ◽  
...  
Keyword(s):  
Liver Failure ◽  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation

scholarly journals Novel compound mutations in the mitochondrial translation elongation factor (TSFM) gene cause severe cardiomyopathy with myocardial fibro-adipose replacement

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Elena Perli ◽  
Annalinda Pisano ◽  
Ruth I. C. Glasgow ◽  
Miriam Carbo ◽  
Steven A. Hardy ◽  
...  
Keyword(s):  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation

Tomato EF-Tsmt, a functional mitochondrial translation elongation factor from higher plants

2003 ◽  
Vol 53 (3) ◽  
pp. 411-422 ◽  
Author(s):  
Mohamed Benichou ◽  
Zhengguo Li ◽  
Barthélémy Tournier ◽  
Ana Chaves ◽  
Hicham Zegzouti ◽  
...  
Keyword(s):  
Higher Plants ◽  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation

scholarly journals Autophagy-competent mitochondrial translation elongation factor TUFM inhibits caspase-8-mediated apoptosis

2021 ◽  
Author(s):  
Chang-Yong Choi ◽  
Mai Tram Vo ◽  
John Nicholas ◽  
Young Bong Choi
Keyword(s):  
Molecular Mechanisms ◽  
Mitochondrial Dynamics ◽  
Elongation Factor ◽  
Productive Infection ◽  
Caspase 8 ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation ◽  
Cell Functions ◽  
Induced Apoptosis

AbstractMitochondria support multiple cell functions, but an accumulation of dysfunctional or excessive mitochondria is detrimental to cells. We previously demonstrated that a defect in the autophagic removal of mitochondria, termed mitophagy, leads to the acceleration of apoptosis induced by herpesvirus productive infection. However, the exact molecular mechanisms underlying activation of mitophagy and regulation of apoptosis remain poorly understood despite the identification of various mitophagy-associated proteins. Here, we report that the mitochondrial translation elongation factor Tu, a mitophagy-associated protein encoded by the TUFM gene, locates in part on the outer membrane of mitochondria (OMM) where it acts as an inhibitor of altered mitochondria-induced apoptosis through its autophagic function. Inducible depletion of TUFM potentiated caspase-8-mediated apoptosis in virus-infected cells with accumulation of altered mitochondria. In addition, TUFM depletion promoted caspase-8 activation induced by treatment with TNF-related apoptosis-inducing ligand in cancer cells, potentially via dysregulation of mitochondrial dynamics and mitophagy. Importantly, we revealed the existence of and structural requirements for autophagy-competent TUFM on the OMM; the GxxxG motif within the N-terminal mitochondrial targeting sequences of TUFM was required for self-dimerization and mitophagy. Furthermore, we found that autophagy-competent TUFM was subject to ubiquitin-proteasome-mediated degradation but stabilized upon mitophagy or autophagy activation. Moreover, overexpression of autophagy-competent TUFM could inhibit caspase-8 activation. These studies extend our knowledge of mitophagy regulation of apoptosis and could provide a novel strategic basis for targeted therapy of cancer and viral diseases.

Expansion of the known phenotype for mitochondrial translation elongation factor G1 (EGF1) due to GFM1 mutations

2014 ◽  
Vol 47 (15) ◽  
pp. 144
Author(s):  
Enas N. Nasr ◽  
Soumya Mikkilineni ◽  
Stacy Hewson ◽  
Susan Blaser ◽  
Annette Feigenbaum ◽  
...  
Keyword(s):  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation

scholarly journals Distinct Clinical Phenotypes Associated with a Mutation in the Mitochondrial Translation Elongation Factor EFTs

2006 ◽  
Vol 79 (5) ◽  
pp. 869-877 ◽  
Author(s):  
Jan A.M. Smeitink ◽  
Orly Elpeleg ◽  
Hana Antonicka ◽  
Heleen Diepstra ◽  
Ann Saada ◽  
...  
Keyword(s):  
Elongation Factor ◽  
Translation Elongation Factor ◽  
Mitochondrial Translation ◽  
Translation Elongation ◽  
Clinical Phenotypes
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