Recombinant Mouse NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (Ndufs4)

1. the partial absence of complex I sensitizes the myocardium towards ischemia reperfusion injury and that the main source of reactive oxygen species following reperfusion is complex III. PMID: 29501746
2. Excitatory synaptic transmission in the parietal association cortex in slices from Ndufs4(KO) animals was hypersensitive to isoflurane compared to control slices. We identified a direct neural circuit between the parietal association cortex and the central thalamus, consistent with a model in which isoflurane sensitivity is mediated by a thalamic signal relayed through excitatory synapses to the parietal association cort PMID: 29136012
3. Study showed that the earliest cell loss in complex 1-deficient Ndufs4 mice retinas is retinal bipolar cells at p20, followed by Starburst Amacrine Cells at p24, that precede a rise in inflammatory molecules at p30 and retinal ganglion cell death at p42; results could suggest a mechanism in which the death of bipolar and amacrine cells incite an inflammatory wave that ultimately results in retinal ganglion cell loss. PMID: 28027875
4. NDUFS4 deletion affected gene expression following neural differentiation and the potential of the cells to generate beating embryoid bodies. PMID: 26608563
5. Using the Ndufs4 knockout (Ndufs4 KO) mouse, a model of Leigh syndrome, we demonstrate for the first time that protein succination is increased in the brainstem (BS), particularly in the vestibular nucleus. PMID: 26450614
6. Locally insufficient respiration capacity of the nerve terminals may drive focal neurodegeneration in the Ndufs4 knockout mouse model of the Leigh syndrome. PMID: 26824698
7. This study demonstrated that Genetic reduction of Ndufs4 function does not lead to loss of dopamine neurons in vivo. PMID: 26070241
8. Reduced adolescent-age spatial learning ability is associated with elevated juvenile-age superoxide levels in complex I mouse mutants. PMID: 25853418
9. Mitochondrial complex I dysfunction in the retina, present in Ndufs4 KO mouse, triggers an innate immune and inflammatory response that results in loss of retinal ganglion cell function and death. PMID: 25652399
10. Global Ndufs4 deletion causes systemic inflammation and osteopetrosis. PMID: 25130399
11. Complex I deficiency due to selective loss of Ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy. PMID: 24705922
12. deletion of Ndufs4 results in a significant loss of complex I-supported respiration in the heart, which is well tolerated with no major changes of cardiac function, energetics, and longevity of the mice under unstressed conditions. PMID: 23931755
13. Oxidation of the reactive oxygen species sensor hydroethidium was increased and mitochondria were less branched and/or shorter in NDUFS4(-/-) fibroblasts. PMID: 23234723
14. the NDUFS4 subunit is of key importance in CI stabilization PMID: 22430089
15. results demonstrate that the loss of Ndufs4 causes only a mild complex I deficiency in vivo, which in dopamine neurons nevertheless leads to alterations that may play a causative or contributing role in the pathophysiology of late onset Parkinson's disease PMID: 22090423
16. 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. PMID: 22535952
17. Tthe absence of NDUFS4 in different mouse tissues results in decreased activity and stability of mitochondrial complex I. PMID: 21965299
18. To explore the lethal, ataxic phenotype of complex I deficiency in Ndufs4 knockout mice, we inactivated Ndufs4 selectively in neurons and glia, resulting in progressive encephalopathy resembling Leigh syndrome. PMID: 20534480
19. CI fails to assemble properly or is unstable without NDUFS4 PMID: 18396137
20. In this study, Ndufs4-/- mice was used as a genetic model to study mitochondrial complex I deficiency and dopaminergic neuron death. PMID: 18812510

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KEGG: mmu:17993

STRING: 10090.ENSMUSP00000022286

UniGene: Mm.253142