Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
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Electron Transport Disturbances and Neurodegeneration: From Albert Szent-Györgyi's Concept (Szeged) till Novel Approaches to Boost Mitochondrial BioenergeticsMouse models of Parkinson's disease associated with mitochondrial dysfunctionMitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouseAmmonium accumulation and cell death in a rat 3D brain cell model of glutaric aciduria type ITissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathyProteomic 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) genePartial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.Lack of GPR88 enhances medium spiny neuron activity and alters motor- and cue-dependent behaviorsA defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions.Sequencing and comparative genomic analysis of 1227 Felis catus cDNA sequences enriched for developmental, clinical and nutritional phenotypes.Transient activation of specific neurons in mice by selective expression of the capsaicin receptor.A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome.mTOR inhibition alleviates mitochondrial disease in a mouse model of Leigh syndrome.Respiratory chain deficiency in aged spinal motor neurons.Glia are critical for the neuropathology of complex I deficiency in DrosophilaPARP inhibition delays progression of mitochondrial encephalopathy in mice.Altered anesthetic sensitivity of mice lacking Ndufs4, a subunit of mitochondrial complex I.Gene expression and functional annotation of the human ciliary body epithelia.Glial lipid droplets and ROS induced by mitochondrial defects promote neurodegeneration.The genetics of Leigh syndrome and its implications for clinical practice and risk managementNeuronal and astrocyte dysfunction diverges from embryonic fibroblasts in the Ndufs4fky/fky mouse.The role of astrocyte mitochondria in differential regional susceptibility to environmental neurotoxicants: tools for understanding neurodegenerationMitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors.Hypothalamic mitochondrial dysfunction associated with anorexia in the anx/anx mouse.Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models.Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons.Neuropathologic Characterization of Pontocerebellar Hypoplasia Type 6 Associated With Cardiomyopathy and Hydrops Fetalis and Severe Multisystem Respiratory Chain Deficiency due to Novel RARS2 Mutations.Modeling mitochondrial dysfunctions in the brain: from mice to men.Dose-dependent effects of mTOR inhibition on weight and mitochondrial disease in miceMitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies.Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse BrainGenetic reduction of mitochondrial complex I function does not lead to loss of dopamine neurons in vivoComplex I dysfunction underlies the glycolytic switch in pulmonary hypertensive smooth muscle cells.Fatal breathing dysfunction in a mouse model of Leigh syndromeBehavioral Phenotyping of Murine Disease Models with the Integrated Behavioral Station (INBEST).Mouse models of mitochondrial complex I dysfunctionIncreased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia.Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome.Hypoxia as a therapy for mitochondrial disease.
P2860
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P2860
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
description
2010 nî lūn-bûn
@nan
2010 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@ast
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@en
type
label
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@ast
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@en
prefLabel
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@ast
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@en
P2860
P50
P356
P1476
Complex I deficiency due to lo ...... thy resembling Leigh syndrome.
@en
P2093
Raj P Kapur
Richard D Palmiter
P2860
P304
10996-11001
P356
10.1073/PNAS.1006214107
P407
P577
2010-06-01T00:00:00Z