In-gel activity shows that amc11 accumulates a sub-complex diagnostic of an assembly defect in the membrane arm of CI. The mutation in amc11 was mapped to a novel gene encoding a unique low complexity protein with no conserved domains. The role of this AMC11 locus in CI assembly was ...
Statistical testing performed by gene set enrichment analysis84, FDR < 0.0001. NES normalized enrichment score. Full size image Mitochondrially encoded complex I genes and IDH1/2 mutations We next used data from an independent AML cohort of 124 de novo cases from Stanford Hospital for which ...
Mitochondrial complex I powers ATP synthesis by oxidative phosphorylation, exploiting the energy from ubiquinone reduction by NADH to drive protons across the energy-transducing inner membrane. Recent cryo-EM analyses of mammalian and yeast complex I have revolutionized structural and mechanistic knowledge ...
The NDUFV1 knockdown-induced blockage of insulin signaling was released by protein-tyrosine phosphatase 1B knockdown in C2C12 myotubes, and we found that NDUFV1 or SIRT1 knockdown did not affect mitochondria biogenesis during C2C12 myogenesis. Based on these data, we can conclude that complex I ...
These results suggest that screening for complex I nuclear gene mutations is of particular interest in patients with complex I deficiency, even when normal respiratory-chain–enzyme activities in cultured fibroblasts are observed.Previous article in issue Next article in issue...
As in other angiosperm mitochondrial genomes, most of the known genes encode for proteins of the electron transport chain, with 9 subunits of complex I (nad1 2 3 4 4 L 5 6 7 and 9), one subunit of complex III (cob), three subunits of complex IV (cox1 2 and 3), five subunits...
The gene order is canonical, but two unusual and large non-coding regions were observed in sites of abrupt shift of the coding polarity. Such intergenic spacers may contain the regulatory signals involved in the replication and/or transcription of the mtDNA, although additional data will be ...
First molecular data on the human roundworm Ascaris lumbricoides species complex from the Bronze and Iron Age in Hallstatt, Austria cytochrome c oxidase subunit 1 gene (cox1), the mitochondrial cytochrome B gene (cytB) and the mitochondrial NADH dehydrogenase subunit 1 gene (nadh1)... E Barsch...
we next focused on genes related to the inflammasome32and the response to ROS. We found that the expression of genes involved in the NLRP3 inflammasome complex (such asNlrp3andDhx33), the antioxidant response (for example,Cybb,Txn1andSod1) and glutathione synthesis/reduction (Gsr)26,33was ...
In summary, employing the mitochondrial complex-1 inhibitor rotenone, we observed an interesting phenomenon, in which inhibition of mitochondrial complex-1 selectively reversed the obstruction-induced downregulation of NKCC2 and ENaCα in kidneys. The distinct responses of sodium transporters to ...