NDUFS4: creation of a mouse model mimicking a Complex I disorder. - Wikidata - wikimedia - TriplyDB

NDUFS4: creation of a mouse model mimicking a Complex I disorder.

NDUFS4: creation of a mouse model mimicking a Complex I disorder.

http://www.wikidata.org/entity/Q37440629

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Complex I deficiency due to selective loss of Ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy Cytoprotection by the modulation of mitochondrial electron transport chain: the emerging role of mitochondrial STAT3 Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) gene Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.Mitochondrial complex I deficiency leads to the retardation of early embryonic development in Ndufs4 knockout mice.D-galactose effectiveness in modeling aging and therapeutic antioxidant treatment in mice.Mitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors.The permeability transition pore controls cardiac mitochondrial maturation and myocyte differentiation.Initiation of electron transport chain activity in the embryonic heart coincides with the activation of mitochondrial complex 1 and the formation of supercomplexes.Animal models of human mitochondrial DNA mutations.Modeling mitochondrial dysfunctions in the brain: from mice to men.Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice Mouse models of mitochondrial complex I dysfunction Chromophore-Assisted Light Inactivation of Mitochondrial Electron Transport Chain Complex II in Caenorhabditis elegans.In vivo cardioprotection by S-nitroso-2-mercaptopropionyl glycine Research on plants for the understanding of diseases of nuclear and mitochondrial origin.Mitochondrial network in the heart.Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice.Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

http://www.wikidata.org/entity/Q37440629

description

article científic

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article scientifique

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articolo scientifico

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artigo científico

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bilimsel makale

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scientific article published on 11 February 2009

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vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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prefLabel

NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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J.MITO.2009.02.001

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NDUFS4: creation of a mouse model mimicking a Complex I disorder.

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Carl A Pinkert

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Christopher A Ingraham

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Jolanta Skalska

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Lindsay S Burwell

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Paul S Brookes

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Robert L Howell

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Shey-Shing Sheu

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P2860

A nonsense mutation in the NDUFS4 gene encoding the 18 kDa (AQDQ) subunit of complex I abolishes assembly and activity of the complex in a patient with Leigh-like syndrome

Late-onset Leigh syndrome in a patient with mitochondrial complex I NDUFS8 mutations

Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I

Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit.

Pathological mutations of the human NDUFS4 gene of the 18-kDa (AQDQ) subunit of complex I affect the expression of the protein and the assembly and function of the complex

Constitutive knockout of Surf1 is associated with high embryonic lethality, mitochondrial disease and cytochrome c oxidase deficiency in mice

The energy-transducing NADH: quinone oxidoreductase, complex I

Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.

High throughput two-dimensional blue-native electrophoresis: a tool for functional proteomics of mitochondria and signaling complexes.

Mitochondrial dysfunction in cardiac ischemia-reperfusion injury: ROS from complex I, without inhibition.

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