LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs
Regulation of mtDNA expression is critical for maintaining cellular energy homeostasis and
may, in principle, occur at many different levels. The leucine‐rich pentatricopeptide repeat …
may, in principle, occur at many different levels. The leucine‐rich pentatricopeptide repeat …
[PDF][PDF] Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome
MD Metodiev, N Lesko, CB Park, Y Cámara, Y Shi… - Cell metabolism, 2009 - cell.com
The 3′ end of the rRNA of the small ribosomal subunit contains two extremely highly
conserved dimethylated adenines. This modification and the responsible methyltransferases …
conserved dimethylated adenines. This modification and the responsible methyltransferases …
[PDF][PDF] MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome
Y Cámara, J Asin-Cayuela, CB Park, MD Metodiev… - Cell metabolism, 2011 - cell.com
Precise control of mitochondrial DNA gene expression is critical for regulation of oxidative
phosphorylation capacity in mammals. The MTERF protein family plays a key role in this …
phosphorylation capacity in mammals. The MTERF protein family plays a key role in this …
Variable and Tissue-Specific Subunit Composition of Mitochondrial m-AAA Protease Complexes Linked to Hereditary Spastic Paraplegia
M Koppen, MD Metodiev, G Casari… - … and cellular biology, 2007 - Taylor & Francis
The m-AAA protease, an ATP-dependent proteolytic complex in the mitochondrial inner
membrane, controls protein quality and regulates ribosome assembly, thus exerting …
membrane, controls protein quality and regulates ribosome assembly, thus exerting …
[PDF][PDF] Mutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathy
R Kopajtich, TJ Nicholls, J Rorbach… - The American Journal of …, 2014 - cell.com
Respiratory chain deficiencies exhibit a wide variety of clinical phenotypes resulting from
defective mitochondrial energy production through oxidative phosphorylation. These defects …
defective mitochondrial energy production through oxidative phosphorylation. These defects …
[PDF][PDF] Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome
The synthesis of all 13 mitochondrial DNA (mtDNA)-encoded protein subunits of the human
oxidative phosphorylation (OXPHOS) system is carried out by mitochondrial ribosomes …
oxidative phosphorylation (OXPHOS) system is carried out by mitochondrial ribosomes …
[PDF][PDF] Recessive mutations in TRMT10C cause defects in mitochondrial RNA processing and multiple respiratory chain deficiencies
Mitochondrial disorders are clinically and genetically diverse, with mutations in
mitochondrial or nuclear genes able to cause defects in mitochondrial gene expression …
mitochondrial or nuclear genes able to cause defects in mitochondrial gene expression …
[HTML][HTML] MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals
Regulation of mitochondrial DNA (mtDNA) expression is critical for the control of oxidative
phosphorylation in response to physiological demand, and this regulation is often impaired …
phosphorylation in response to physiological demand, and this regulation is often impaired …
[PDF][PDF] A common variant in TFB1M is associated with reduced insulin secretion and increased future risk of type 2 diabetes
T Koeck, AH Olsson, MD Nitert, VV Sharoyko… - Cell metabolism, 2011 - cell.com
Summary Type 2 diabetes (T2D) evolves when insulin secretion fails. Insulin release from
the pancreatic β cell is controlled by mitochondrial metabolism, which translates fluctuations …
the pancreatic β cell is controlled by mitochondrial metabolism, which translates fluctuations …
[PDF][PDF] Impaired transferrin receptor palmitoylation and recycling in neurodegeneration with brain iron accumulation
A Drecourt, J Babdor, M Dussiot, F Petit… - The American Journal of …, 2018 - cell.com
Neurodegeneration with brain iron accumulation (NBIA) is a genetically heterogeneous
condition characterized by progressive dystonia with iron accumulation in the basal ganglia …
condition characterized by progressive dystonia with iron accumulation in the basal ganglia …