Article

Iron Status Influences Mitochondrial Disease Progression in Complex I-Deficient Mice

2021-09-29

Abstract excerpt

Mitochondrial dysfunction caused by aberrant Complex I assembly and reduced activity of the electron transport chain is pathogenic in many genetic and age-related diseases. Mice missing the Complex I subunit NADH dehydrogenase [ubiquinone] iron-sulfur protein 4 (NDUFS4) are a leading mammalian model of severe mitochondrial disease that exhibit many characteristic symptoms of Leigh Syndrome including oxidative stre...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 7a0d6ecb-0d76-5751-8723-108bfb63ddc8
DOI: 10.1101/2021.09.29.462431

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Iron Status Influences Mitochondrial Disease Progression in Complex I-Deficient MiceDOI 10.1101/2021.09.29.462431Read

IFNγ modestly contributes to disease progression in the <i>Ndufs4</i> (-/-) model of Leigh syndrome while IP10 is dispensableSemantic proximity Read

Respiratory complex III <sub>2</sub> assembles complex I via toxic intermediate in mitochondrial diseaseSemantic proximity Read

Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.Semantic proximity Read

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.Semantic proximity Read

Peripheral macrophages causally contribute to disease onset and progression in the <i>Ndufs4</i> (KO) model of Leigh syndromeSemantic proximity Read

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.Semantic proximity Read

Emerging Roles of NDUFS8 Located in Mitochondrial Complex I in Different Diseases.Semantic proximity Read

Structural insights into complex I deficiency and assembly from the disease-related <i>ndufs4</i> <sup>-/-</sup> mouseSemantic proximity Read

Impaired Complex I dysregulates neural/glial precursors and corpus callosum development revealing postnatal defects in Leigh Syndrome miceSemantic proximity Read

Defined neuronal populations drive fatal phenotype in Leigh SyndromeSemantic proximity Read

WDR45-related encephalopathy mimicking Leigh syndrome associated with complex I deficiency: a case reportSemantic proximity Read

AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome.Semantic proximity Read