LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

B Ruzzenente, MD Metodiev, A Wredenberg… - The EMBO …, 2012 - embopress.org
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 …

[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 …

[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 …

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 …

[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 …

[PDF][PDF] Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome

NJ Lake, BD Webb, DA Stroud, TR Richman… - The American Journal of …, 2017 - cell.com
The synthesis of all 13 mitochondrial DNA (mtDNA)-encoded protein subunits of the human
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

MD Metodiev, K Thompson, CL Alston… - The American Journal of …, 2016 - cell.com
Mitochondrial disorders are clinically and genetically diverse, with mutations in
mitochondrial or nuclear genes able to cause defects in mitochondrial gene expression …

[HTML][HTML] MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals

A Wredenberg, M Lagouge, A Bratic, MD Metodiev… - PLoS …, 2013 - journals.plos.org
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 …

[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 …

[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 …