MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention

Human Molecular Genetics

Volumn 24, Issue 8, 2015, Pages 2247-2266

  Tischner, Christin ^a   Hofer, Annette ^a   Wulff, Veronika ^a   Stepek, Joanna ^a   Dumitru, Iulia ^a   Becker, Lore ^b,c   Haack, Tobias ^c,d   Kremer, Laura ^c,d   Datta, Alexandre N ^e   Sperl, Wolfgang ^d,f   Floss, Thomas ^c   Wurst, W Wolfgang ^b,c,g,h,i   Chrzanowska Lightowlers, Zofia ^j   De Angelis, Martin Hrabe ^b,c,g,k   Klopstock, Thomas ^b,c,d,h   Prokisch, Holger ^c   Wenz, Tina ^a,d  

^a UNIVERSITY OF COLOGNE   (Germany)

^b LUDWIG MAXIMILIANS UNIVERSITY   (Germany)

^c INSTITUTE OF EPIDEMIOLOGY   (Germany)

^d German Network for Mitochondrial Disorders (MitoNET)   (Germany)

^e UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

^f PARACELSUS MEDICAL UNIVERSITY   (Austria)

^g TECHNICAL UNIVERSITY OF MUNICH   (Germany)

^h GERMAN CENTER FOR NEURODEGENERATIVE DISEASES DZNE   (Germany)

ⁱ MAX PLANCK INSTITUTE OF PSYCHIATRY   (Germany)

^j NEWCASTLE UNIVERSITY   (United Kingdom)

^k GERMAN CENTER FOR DIABETES RESEARCH DZD   (Germany)

more..

Author keywords

[No Author keywords available]

Indexed keywords

CYTOCHROME C OXIDASE; MESSENGER RNA; MITOCHONDRIAL PROTEIN; MITOCHONDRIAL TRANSLATION OPTIMIZATION FACTOR 1; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); TRANSFER RNA; UNCLASSIFIED DRUG; CARRIER PROTEIN; MTO1 PROTEIN, HUMAN; MTO1 PROTEIN, MOUSE;

AEROBIC METABOLISM; ANIMAL TISSUE; ARTICLE; CELL STRESS; CHEMICAL MODIFICATION; COMPLEX FORMATION; CONTROLLED STUDY; ENZYME ACTIVATION; ENZYME ACTIVITY; HUMAN; HUMAN CELL; IN VIVO STUDY; KETOGENIC DIET; MOUSE; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN EXPRESSION; PROTEIN SYNTHESIS; RNA MODIFICATION; RNA TRANSLATION; STEADY STATE; TISSUE SPECIFICITY; UNFOLDED PROTEIN RESPONSE; AMINO ACID SEQUENCE; ANIMAL; ANTIBODY SPECIFICITY; CHEMISTRY; FIBROBLAST; GENETICS; METABOLISM; MITOCHONDRIAL DISEASES; MITOCHONDRION; MOLECULAR GENETICS; SEQUENCE ALIGNMENT;

MAMMALIA; MUS;

AMINO ACID SEQUENCE; ANIMALS; CARRIER PROTEINS; FIBROBLASTS; HUMANS; KETOGENIC DIET; MICE; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MOLECULAR SEQUENCE DATA; ORGAN SPECIFICITY; OXIDATIVE PHOSPHORYLATION; PROTEIN BIOSYNTHESIS; RNA, TRANSFER; SEQUENCE ALIGNMENT;

EID: 84926472434     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddu743     Document Type: Article

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