Respiratory complex III dysfunction in humans and the use of yeast as a model organism to study mitochondrial myopathy and associated diseases

Biochimica et Biophysica Acta - Bioenergetics

Volumn 1827, Issue 11-12, 2013, Pages 1346-1361

  Meunier, B ^a   Fisher, N ^b   Ransac, S ^c   Mazat, J P ^c   Brasseur, G ^d  

^a CENTRE DE GÉNÉTIQUE MOLÉCULAIRE   (France)

^b MICHIGAN STATE UNIVERSITY   (United States)

^c Institut de Biochimie et Genetique Cellulaires   (France)

^d AIX MARSEILLE UNIVERSITÉ   (France)

Author keywords

bc1 complex; BCS1 BCS1L; Complex III mutations; Human myopathy; Mitochondrial cytochrome b; Yeast Saccharomyces cerevisiae

Indexed keywords

ADENOSINE TRIPHOSPHATE; ASCORBIC ACID; BIOMATERIAL; CYTOCHROME B; MENADIONE; MITOCHONDRIAL DNA; OXYGEN; PHOSPHOETHANOLAMINE; PROTON; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; REACTIVE OXYGEN METABOLITE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINIC ACID; SULFUR; UBIQUINOL CYTOCHROME C REDUCTASE; UBIQUINONE;

AMINO TERMINAL SEQUENCE; AMINOACIDURIA; ATAXIA; CARDIOMYOPATHY; CATARACT; CHOLESTASIS; COMPLEX III DEFICIENCY; DEMENTIA; DISEASE ASSOCIATION; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ELECTRON; EMBRYO DEVELOPMENT; ENZYME ACTIVE SITE; EUKARYOTE; FATTY LIVER; GENE EXPRESSION; GENE MUTATION; GENOME; GRACILE SYNDROME; HEART ARREST; HETEROPLASMY; HUMAN; IRON OVERLOAD; KIDNEY TUBULE NECROSIS; LACTIC ACIDOSIS; LEBER HEREDITARY OPTIC NEUROPATHY; LEUKOCYTE; LIVER MITOCHONDRION; MELAS SYNDROME; MENTAL DEFICIENCY; MENTAL DISEASE; MISSENSE MUTATION; MITOCHONDRIAL ENCEPHALOMYOPATHY; MITOCHONDRIAL GENOME; MITOCHONDRIAL MEMBRANE; MITOCHONDRIAL MYOPATHY; MULTIPLE ORGAN FAILURE; MUSCLE MITOCHONDRION; MUSCLE WEAKNESS; NEUROLOGIC DISEASE; NEUROMUSCULAR DISEASE; NONHUMAN; NONSENSE MUTATION; OXIDATIVE PHOSPHORYLATION; PERCEPTION DEAFNESS; PHENOTYPE; PRIORITY JOURNAL; PROTON MOTIVE FORCE; RESPIRATORY CHAIN; RETINITIS PIGMENTOSA; REVIEW; SACCHAROMYCES CEREVISIAE; SENSORIMOTOR NEUROPATHY; SKELETAL MUSCLE;

A.A.; AMINO-ACID; BC(1) COMPLEX; BCS1 BCS1L; CI-V; COMPLEX I-V; COMPLEX III MUTATIONS; CYT; CYTOCHROME; HAEM B(H); HAEM B(L); HIGH MID-POINT POTENTIAL CYT B HAEM; HUMAN MYOPATHY; ISP; LEBER HEREDITARY OPTIC NEUROPATHY; LHON; LOW MID-POINT POTENTIAL CYT B HAEM; MELAS; MITOCHONDRIAL CYTOCHROME B; MITOCHONDRIAL ENCEPHALOMYOPATHY LACTIC ACIDOSIS AND STROKE-LIKE EPISODES; Q(I); Q(O); QUINOL OXIDISING SITE ON THE POSITIVE SIDE OF THE MEMBRANE; QUINONE REDUCTASE SITE ON THE NEGATIVE SIDE OF THE MEMBRANE; REACTIVE OXYGEN SPECIES; RIESKE OR IRON SULFUR PROTEIN; ROS; SUB; SUBUNIT; YEAST SACCHAROMYCES CEREVISIAE;

ELECTRON TRANSPORT; ELECTRON TRANSPORT COMPLEX III; HUMANS; MITOCHONDRIAL MYOPATHIES; MODELS, MOLECULAR; MUTATION; PROTEIN CONFORMATION; PROTEIN SUBUNITS; SACCHAROMYCES CEREVISIAE; SACCHAROMYCES CEREVISIAE PROTEINS;

EUKARYOTA; PROKARYOTA; SACCHAROMYCES CEREVISIAE;

EID: 84884675005     PISSN: 00052728     EISSN: 18792650     Source Type: Journal    
DOI: 10.1016/j.bbabio.2012.11.015     Document Type: Review

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