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Behavioural Brain Research, ISSN 0166-4328, 01/2016, Volume 296, pp. 191 - 198
Leigh disease (LD) is one of the clinical phenotypes of mitochondrial OXPHOS disorders and also known as sub-acute necrotizing encephalomyelopathy. The disease... 
Ndufs4 knockout mice | Mitochondrial complex I deficiency | CatWalk system | Gait analysis | DIAGNOSIS | NDUFS4 | BEHAVIORAL SCIENCES | ATAXIA | NEUROSCIENCES | COMPLEX-I DEFICIENCY | Medicine, Experimental | Walking | Medical research | Animal behavior | Analysis | Index Medicus
Journal Article
Journal Article
Journal Article
ISSN 0969-7128, 2017
Leigh syndrome (LS) is the most common infantile mitochondrial encephalopathy. No treatment is currently available for this condition. Mice lacking... 
mouse models | experimental therapy | Leigh syndrome | AAV | gene therapy | Ndufs4 | Mitochondrial disease
Journal Article
Science, ISSN 0036-8075, 12/2013, Volume 342, Issue 6165, pp. 1524 - 1528
Journal Article
Journal Article
JOURNAL OF BIOLOGICAL CHEMISTRY, ISSN 0021-9258, 08/2019, Volume 294, Issue 33, pp. 12432 - 12443
Mitochondrial dysfunction is an important cause of heritable vision loss. Mutations affecting mitochondrial bioenergetics may lead to isolated vision loss or... 
CELLS | TRANSLOCATION | MITOCHONDRIAL | neurological disease | BIOCHEMISTRY & MOLECULAR BIOLOGY | photoreceptor | phototransduction | CHILDREN | optic atrophy | TRANSDUCIN | gene knockout | transgenic mice | NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4) | retina | GLUCOSE | MICE | HEREDITARY OPTIC NEUROPATHY
Journal Article
Annual Review of Biophysics, ISSN 1936-122X, 5/2019, Volume 48, Issue 1, pp. 165 - 184
Journal Article
SCIENTIFIC REPORTS, ISSN 2045-2322, 02/2019, Volume 9, Issue 1, pp. 3073 - 10
Leigh syndrome is a mitochondrial disease characterized by neurological disorders, metabolic abnormality and premature death. There is no cure for Leigh... 
GLUTAMATE-DEHYDROGENASE | NICOTINAMIDE RIBOSIDE | LIFE-SPAN | LEIGH-SYNDROME | ACETYLATION | MULTIDISCIPLINARY SCIENCES | MOUSE MODEL | NDUFS4 | HYPOXIA | BRAIN | COMPLEX-I DEFICIENCY | NADPH | Phenotypes | Therapeutic applications | Skeletal muscle | NAD | Neurological diseases | Mitochondria | Life span | Rodents | Nicotinamide | Supplementation | Acidosis | Lactic acidosis
Journal Article
Cell Reports, ISSN 2211-1247, 07/2017, Volume 20, Issue 1, pp. 264 - 278
The flight muscles of are highly enriched with mitochondria, but the mechanism by which mitochondrial complex I (CI) is assembled in this tissue has not been... 
complex I biogenesis/assembly | mitochondria | Drosophila | CHAIN | LONGEVITY | PROTEIN | ARCHITECTURE | NEUROSPORA MITOCHONDRIA | SUBUNITS | MUTATIONS | NDUFS4 GENE | EXPRESSION | DEFICIENCY | CELL BIOLOGY
Journal Article