Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage

scientific article

Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1093/HMG/DDT526
P932PMC publication ID3929083
P698PubMed publication ID24154540
P5875ResearchGate publication ID258040414

P2093author name stringTina Wenz
Carlos T Moraes
Francisca Diaz
Susana Peralta
Alessandra Torraco
Sofia Garcia
P2860cites workCoupling mitochondrial respiratory chain to cell death: an essential role of mitochondrial complex I in the interferon-beta and retinoic acid-induced cancer cell deathQ24296337
Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex IQ24304454
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Crystal structure of the entire respiratory complex IQ27676412
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The electron transfer flavoprotein: Ubiquinone oxidoreductasesQ28295323
Mitochondrial DNA mutations induce mitochondrial dysfunction, apoptosis and sarcopenia in skeletal muscle of mitochondrial DNA mutator miceQ28474793
2-Deoxy-D-glucose treatment induces ketogenesis, sustains mitochondrial function, and reduces pathology in female mouse model of Alzheimer's diseaseQ28478912
Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathyQ28505379
mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcriptionQ28512464
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) geneQ28579972
Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblastsQ28585444
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Chronic systemic pesticide exposure reproduces features of Parkinson's diseaseQ29614763
Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian agingQ29616056
Molecular Pathways of Neurodegeneration in Parkinson's DiseaseQ29619903
A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions.Q30416049
High frequency of mitochondrial complex I mutations in Parkinson's disease and agingQ30434711
Mitochondrial ND5 mutations in idiopathic Parkinson's diseaseQ30435386
Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's diseaseQ30443526
Ketogenic diet slows down mitochondrial myopathy progression in miceQ43157369
Analysis of the subunit composition of complex I from bovine heart mitochondriaQ44367201
Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's diseaseQ45294442
Functional modules and structural basis of conformational coupling in mitochondrial complex I.Q45730820
Capacity for substrate utilization in oxidative metabolism by neurons, astrocytes, and oligodendrocytes from developing brain in primary cultureQ48237711
Role of carnitine palmitoyltransferase I in the control of ketogenesis in primary cultures of rat astrocytesQ48388052
Superoxide production is inversely related to complex I activity in inherited complex I deficiency.Q50471908
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.Q50488580
Formation of 8-hydroxyguanine residues in DNA by X-irradiationQ70754172
The Charge Heterogeneity of the Mitochondrial Acetyl-CoA Acetyltransferase from Rat LiverQ72521322
CaMKIIalpha-Cre transgene expression and recombination patterns in the mouse brainQ73473631
Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathyQ30484862
Gene-trap mutagenesis: past, present and beyondQ30728811
Mitochondrial DNA damage in a mouse model of Alzheimer's disease decreases amyloid beta plaque formationQ33608218
Neurotoxic in vivo models of Parkinson's disease recent advances.Q33708283
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndromeQ33934412
Bovine complex I is a complex of 45 different subunits.Q33999203
The harlequin mouse mutation downregulates apoptosis-inducing factorQ34152182
Definition of the nuclear encoded protein composition of bovine heart mitochondrial complex I. Identification of two new subunits.Q34154561
Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species productionQ34212784
Mitochondria and degenerative disordersQ34386737
Mitochondrial dysfunction during hypoxia/reoxygenation and its correction by anaerobic metabolism of citric acid cycle intermediatesQ35082127
Ketone body synthesis in the brain: possible neuroprotective effectsQ35652465
Novel role for mitochondria: protein kinase Ctheta-dependent oxidative signaling organelles in activation-induced T-cell deathQ35856966
D-beta-hydroxybutyrate rescues mitochondrial respiration and mitigates features of Parkinson diseaseQ35919815
The epidemiology of mitochondrial disorders--past, present and futureQ35970168
Fatal breathing dysfunction in a mouse model of Leigh syndromeQ36070987
Cerebral ketone body metabolismQ36118288
Mouse mtDNA mutant model of Leber hereditary optic neuropathy.Q36471358
The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?Q37134820
Electrophoresis techniques to investigate defects in oxidative phosphorylationQ37307214
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.Q37428322
NDUFS4: creation of a mouse model mimicking a Complex I disorderQ37440629
Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation.Q37608294
AIF deficiency compromises oxidative phosphorylationQ37671807
Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.Q37682674
Mitochondrial disorders caused by mutations in respiratory chain assembly factorsQ37890682
Understanding mitochondrial complex I assembly in health and diseaseQ37933572
Assembly factors as a new class of disease genes for mitochondrial complex I deficiency: cause, pathology and treatment optionsQ37950745
Mitochondrial complex I deficiency of nuclear origin II. Non-structural genesQ37958285
Mitochondrial complex I deficiency of nuclear origin I. Structural genesQ37964862
Which way does the citric acid cycle turn during hypoxia? The critical role of α-ketoglutarate dehydrogenase complex.Q38078892
Acetyl-CoA acetyltransferase from bovine liver mitochondria Molecular properties of multiple formsQ39225716
Mechanism of action of a wound-induced ω-hydroxyfatty acid:NADP oxidoreductase isolated from potato tubers (Solanum tuberosum L)Q39236604
Inhibition of complex I of the electron transport chain causes O2-. -mediated mitochondrial outgrowth.Q39704890
Respiratory active mitochondrial supercomplexes.Q39913363
Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using polarography and spectrophotometric enzyme assaysQ41861486
Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using blue native gel electrophoresisQ42606879
P433issue6
P921main subjectSuccinate dehydrogenase complex, subunit B, iron sulfur (Ip)Q14916128
Cytochrome c oxidase subunit IQ21984161
Ubiquinol cytochrome c reductase core protein 2Q21987463
NADH:ubiquinone oxidoreductase subunit A5Q21988742
NADH:ubiquinone oxidoreductase core subunit S4Q21988755
NADH:ubiquinone oxidoreductase subunit B8Q21988757
NADH:ubiquinone oxidoreductase subunit A9Q21988760
NADH:ubiquinone oxidoreductase core subunit S3Q21988764
P304page(s)1399-1412
P577publication date2013-10-23
P1433published inHuman Molecular GeneticsQ2720965
P1476titlePartial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage
P478volume23

Reverse relations

cites work (P2860)
Q32884468Accessory subunits are integral for assembly and function of human mitochondrial complex I.
Q104479773Adult mesenchymal stem cell ageing interplays with depressed mitochondrial Ndufs6
Q35647954Cardiac metabolic pathways affected in the mouse model of barth syndrome
Q47979884Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice
Q35190973Mitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors
Q48098220Mitochondrial retrograde signaling in the nervous system
Q35517850Moderate hypoxia induces β-cell dysfunction with HIF-1-independent gene expression changes
Q35733751Modulation of mitochondrial complex I activity averts cognitive decline in multiple animal models of familial Alzheimer's Disease
Q92511662Myopathy reversion in mice after restauration of mitochondrial complex I
Q36573120PPARα modulates gene expression profiles of mitochondrial energy metabolism in oral tumorigenesis
Q90273036Prediction of tumor location in prostate cancer tissue using a machine learning system on gene expression data
Q99634865Proteomics Reveals the Potential Protective Mechanism of Hydrogen Sulfide on Retinal Ganglion Cells in an Ischemia/Reperfusion Injury Animal Model
Q38861730Review: Central nervous system involvement in mitochondrial disease
Q37722131Systematic analysis of microarray datasets to identify Parkinson's disease‑associated pathways and genes
Q36506026The REST remodeling complex protects genomic integrity during embryonic neurogenesis.

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