222 results on '"Fernandez-Vizarra, Erika"'
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2. Residual Complex I activity and amphidirectional Complex II operation support glutamate catabolism through mtSLP in anoxia
3. Sub-organellar mitochondrial hydrogen peroxide observed using a SNAP tag targeted coumarin-based fluorescent reporter
4. Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies
5. The striking differences in the bioenergetics of brain and liver mitochondria are enhanced in mitochondrial disease
6. Cooperative assembly of the mitochondrial respiratory chain
7. Two independent respiratory chains adapt OXPHOS performance to glycolytic switch
8. SILAC-based complexome profiling dissects the structural organization of the human respiratory supercomplexes in SCAFIKO cells
9. CEDAR, an online resource for the reporting and exploration of complexome profiling data
10. Duplexing complexome profiling with SILAC to study human respiratory chain assembly defects
11. NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate
12. CG7630 is the Drosophila melanogaster homolog of the cytochrome c oxidase subunit COX7B
13. Biallelic mutations in NDUFA8 cause complex I deficiency in two siblings with favorable clinical evolution
14. A homozygous MRPL24 mutation causes a complex movement disorder and affects the mitoribosome assembly
15. COA8 is a COX10-binding protein involved in the early biogenesis of cytochrome c oxidase
16. Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: Switch from RET and ROS to FET
17. Blue-Native Electrophoresis to Study the OXPHOS Complexes
18. The levels and activation state of the pyruvate dehydrogenase complex modulate the SCAFI-dependent organization of the mitochondrial respiratory chain
19. TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III
20. Novel mutation in mitochondrial Elongation Factor EF-Tu associated to dysplastic leukoencephalopathy and defective mitochondrial DNA translation
21. Mitochondrial complex I deficiency stratifies idiopathic Parkinson’s disease
22. Exome sequencing coupled with mRNA analysis identifies NDUFAF6 as a Leigh gene
23. Mitochondrially-targeted APOBEC1 is a potent mtDNA mutator affecting mitochondrial function and organismal fitness in Drosophila
24. Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7
25. miR‐181a/b downregulation exerts a protective action on mitochondrial disease models
26. Neuronal complex I deficiency occurs throughout the Parkinson’s disease brain, but is not associated with neurodegeneration or mitochondrial DNA damage
27. Mitochondrial Neurodegeneration: Lessons from Drosophila melanogaster Models
28. APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS
29. LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells
30. Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain
31. Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration
32. Mutant TRIAP1 causes impaired mitochondrial bioenergetics and myopathy
33. Two respiratory chain organizations with distinct bioenergetic properties coexist in human mitochondria
34. Proteomics and gene expression analyses of mitochondria from squalene-treated apoE-deficient mice identify short-chain specific acyl-CoA dehydrogenase changes associated with fatty liver amelioration
35. Partial tandem duplication of mtDNA–tRNA Phe impairs mtDNA translation in late-onset mitochondrial myopathy
36. Tissue-specific differences in mitochondrial activity and biogenesis
37. Measurement of mitochondrial respiratory chain enzymatic activities in Drosophila melanogaster samples
38. Isolation of mitochondria for biogenetical studies: An update
39. Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects
40. Inflammation causes remodeling of mitochondrial cytochrome c oxidase mediated by the bifunctional gene C15orf48
41. Mitochondrial complex III Rieske Fe-S protein processing and assembly
42. Severe Infantile Encephalomyopathy Caused by a Mutation in COX6B1, a Nucleus-Encoded Subunit of Cytochrome C Oxidase
43. FASTKD2 nonsense mutation in an infantile mitochondrial encephalomyopathy associated with cytochrome C oxidase deficiency
44. CEDAR, an online resource for the reporting and exploration of complexome profiling data
45. Infantile encephalopathy and defective mitochondrial DNA translation in patients with mutations of mitochondrial elongation factors EFG1 and EFTu
46. Neural stem cells traffic functional mitochondria via extracellular vesicles
47. Mitochondrial disorders of the OXPHOS system
48. CEDAR, an online resource for the reporting and exploration of complexome profiling data
49. Biogenesis of NDUFS3-less complex I indicates TMEM126A/OPA7 as an assembly factor of the ND4-module
50. Mutation in the MICOS subunit gene APOO (MIC26) associated with an X-linked recessive mitochondrial myopathy, lactic acidosis, cognitive impairment and autistic features
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