Cellular and animal models for mitochondrial complex i deficiency: A focus on the NDUFS4 subunit

IUBMB Life

Volumn 65, Issue 3, 2013, Pages 202-208

  Breuer, Megan E ^a,b   Willems, Peter H G M ^a   Smeitink, Jan A M ^b   Koopman, Werner J H ^a   Nooteboom, Marco ^a,b  

^a RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^b RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

fibroblasts; knockout mouse; Leigh syndrome; neurodegenerative disease; validity

Indexed keywords

REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE);

ANIMAL MODEL; ATAXIA; CELL STIMULATION; COMPLEX I DEFICIENCY; CONSTRUCT VALIDITY; DEATH; DEGENERATIVE DISEASE; DISEASE COURSE; DISEASE MODEL; DNA MODIFICATION; DOPAMINERGIC NERVE CELL; ENCEPHALOMYOPATHY; ENZYME ACTIVITY; FACE VALIDITY; FAILURE TO THRIVE; GENE DELETION; HAIR LOSS; HUMAN; KNOCKOUT MOUSE; LEIGH DISEASE; METABOLIC DISORDER; MITOCHONDRIAL MEMBRANE POTENTIAL; MOTOR DYSFUNCTION; NONHUMAN; NONSENSE MUTATION; ONSET AGE; OXIDATIVE STRESS; PARKINSON DISEASE; POINT MUTATION; PREDICTIVE VALIDITY; PROTON TRANSPORT; PURKINJE CELL; RESPIRATORY FAILURE; REVIEW; SKIN FIBROBLAST; VISUAL IMPAIRMENT;

ANIMALS; CALCIUM; DISEASE MODELS, ANIMAL; ELECTRON TRANSPORT COMPLEX I; EXONS; FIBROBLASTS; GENE EXPRESSION; HUMANS; MICE; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MODELS, BIOLOGICAL; MUTATION; NADH DEHYDROGENASE; OXIDATIVE PHOSPHORYLATION; PROTEIN SUBUNITS;

EID: 84874470617     PISSN: 15216543     EISSN: 15216551     Source Type: Journal    
DOI: 10.1002/iub.1127     Document Type: Review

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