Mutation in MRPS34 Compromises Protein Synthesis and Causes Mitochondrial Dysfunction

Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Mitochondria: Impaired mitochondrial translation in human disease

(2014) Veronika Boczonadi et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Structure of the large ribosomal subunit from human mitochondria

(2014) A. Brown et al.   SCIENCE

Organelle transcriptomes: products of a deconstructed genome

(2013) Ian D Small et al.   CURRENT OPINION IN MICROBIOLOGY

Whole-exome sequencing identifies a mutation in the mitochondrial ribosome protein MRPL44 to underlie mitochondrial infantile cardiomyopathy

(2013) Christopher J Carroll et al.   JOURNAL OF MEDICAL GENETICS

Architecture of the large subunit of the mammalian mitochondrial ribosome

(2013) Basil J. Greber et al.   NATURE

Imaging of neutral lipids by oil red O for analyzing the metabolic status in health and disease

(2013) Annika Mehlem et al.   Nature Protocols

Analysis Tool Web Services from the EMBL-EBI

(2013) Hamish McWilliam et al.   NUCLEIC ACIDS RESEARCH

An RMND1 Mutation Causes Encephalopathy Associated with Multiple Oxidative Phosphorylation Complex Deficiencies and a Mitochondrial Translation Defect

(2012) Alexandre Janer et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Structural aspects of mitochondrial translational apparatus

(2012) Rajendra K Agrawal et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Mitochondrial disorders as windows into an ancient organelle

(2012) Scott B. Vafai et al.   NATURE

Massively parallel sequencing of the mouse exome to accurately identify rare, induced mutations: an immediate source for thousands of new mouse models

(2012) T. D. Andrews et al.   Open Biology

The Human Mitochondrial Transcriptome

(2011) Tim R. Mercer et al.   CELL

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

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation

(2011) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Mutations in C12orf65 in Patients with Encephalomyopathy and a Mitochondrial Translation Defect

(2010) Hana Antonicka et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Interleukin-6-deficient mice develop hepatic inflammation and systemic insulin resistance

(2010) V. B. Matthews et al.   DIABETOLOGIA

Acute liver failure with subsequent cirrhosis as the primary manifestation of TRMU mutations

(2010) Ulrike Schara et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Inventory control: cytochrome c oxidase assembly regulates mitochondrial translation

(2010) David U. Mick et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Pentatricopeptide repeat domain protein 3 associates with the mitochondrial small ribosomal subunit and regulates translation

(2009) Stefan M.K. Davies et al.   FEBS LETTERS

Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells

(2009) Oliver Rackham et al.   NUCLEIC ACIDS RESEARCH

Structure of a mitochondrial ribosome with minimal RNA

(2009) M. R. Sharma et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The effect of mutated mitochondrial ribosomal proteins S16 and S22 on the assembly of the small and large ribosomal subunits in human mitochondria

(2008) Md. Emdadul Haque et al.   MITOCHONDRION