It uncouples oxidative phosphorylation by carrying protons across the mitochondrial membrane, leading to a rapid consumption of energy without generation of ATP.
#F-ATPase: mitochondrial ATP synthase, chloroplast ATP synthase
Alpers' disease is caused by an underlying mitochondrial metabolic defect of POLG,.
This gene encodes an assembly factor for the F(1) component of the mitochondrial ATP synthase.
The cladogram was based on DNA sequences of both nuclear histone H3 and mitochondrial cytochrome-c oxidase I (COI) protein-coding gene regions.
One study showed that mtDNA deletion seen in CPEO patients also had an associated nuclear DNA deletion of the Twinkle gene which encodes specific mitochondrial protein; Twinkle.
Cytoplasmic male sterility is total or partial male sterility in plants as the result of specific nuclear and mitochondrial interactions.
Efrapeptins are peptides made by Tolypocladium that have antifungal, insecticidal, and mitochondrial ATPase inhibitory activities.
Based on the mitochondrial DNA analysis results, many species of Elaphe have been moved to the genera Bogertophis, Gonyosoma, Orthriophis, Pantherophis, Rhinechis, Senticolis, Zamenis and more.
The Warburg hypothesis claims that cancer is primarily caused by dysfunctionality in mitochondrial metabolism, rather than because of uncontrolled growth of cells.
Bryan Sykes in his book Blood of the Isles gives imaginative names to the founders or "clan patriarchs" of major British Y haplogroups, much as he did for mitochondrial haplogroups in his work The Seven Daughters of Eve.
Phylogenetic analysis of the mitochondrial 16S ribosomal RNA gene and a range of other anomuran crustaceans, using Bayesian inference, places this species from the East Scotia Ridge as a sister taxon to Kiwa hirsuta, with a sequence divergence from this species of 6.45%, which is consistent for within-genus divergence in squat lobsters.
Bryan Sykes had claimed there were seven major mitochondrial lineages for modern Europeans but others now put the number at 10-12.
There have been many suggestions to what this might be, the sarcolemmal ATP-sensitive potassium channel, the mitochondrial ATP-sensitive potassium channel, the mitochondrial permeability transition pore, reactive oxygen species generation, chloride channels, the inward rectifier potassium ion channel and connexon 43 related channels.
The Lavasoa dwarf lemur, named for the Lavasoa Mountains in southern Madagascar, became the sixth known species in 2013 when it was differentiated from the furry-eared dwarf lemur (C. crossleyi) through analyses of mitochondrial and nuclear material performed by a team of German and Malagasy researchers.
This has been shown to take place both in the field and in laboratory cultures and has been studied by molecular analysis of sequences of mitochondrial tRNA to identify the taxa involved.
A 2011 analysis by Amy Driskell and colleagues of mitochondrial and nuclear DNA found the Broad-billed Fairywren and Campbell's Fairywren to lie in a clade with the two other monospecific New Guinea genera and not with the other species of Malurus.
In vitro studies suggest that methylene blue might be an effective remedy for both Alzheimer's and Parkinson's disease by enhancing key mitochondrial biochemical pathways.
The NRFs, in turn, activate the mitochondrial transcription factor A (tfam), which is directly responsible for transcribing nuclear-encoded mitochondrial proteins.
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The master regulators of mitochondrial biogenesis appear to be the peroxisome proliferator-activated receptor gamma (PGC) family of transcriptional coactivators, including PGC-1α, PGC-1β, and the PGC-related coactivator, PRC.
There is a mitochondrial outer membrane fusion machinery, composed by proteins of the Fzo1 family in yeast or by MFN1, MFN2 in mammals, and a mitochondrial inner membrane fusion machinery, with the Mgm1 protein in yeast or the equivalent OPA1 in mammals.
Unlike typical mitochondrial diseases caused by mitochondrial DNA (mtDNA) mutations, MNGIE is caused by mutations in the TYMP gene, which encodes the enzyme thymidine phosphorylase.
Mutations in the HADHA and HADHB genes cause mitochondrial trifunctional protein deficiency.
In the pancreas these genes influence expression of, among others, the genes for insulin, the principal glucose transporter (GLUT2), and several proteins involved in glucose and mitochondrial metabolism.
The metaxin gene, which encodes a protein located on the outer membrane of mitochondria, is a component of the mitochondrial protein translocation apparatus.
Subsequently, using DNA from a descendant of Van Zandt, Dr. Odile Loreille, an expert in DNA analysis, was also able to identify the remains using mitochondrial and Y-DNA identification.
Nrf1, together with Nrf2, mediates the biogenomic coordination between nuclear and mitochondrial genomes by directly regulating the expression of several nuclear-encoded ETC proteins, and indirectly regulating the three mitochondrial-encoded COX subunit genes by activating mtTFA, mtTFB1, and mtTFB2.
Noah23 - "Trilateral Damage" from Mitochondrial Blues (2004)
When the inhibition of these is lifted, they result in the translocation of Bax and activation of mitochondrial dysfunction resulting in release of mitochondrial apoptogenic proteins cytochrome c, SMAC, and apoptosis-inducing factor (AIF) leading to caspase activation and cell death.
Recent maternal mitochondrial DNA analysis suggests that Polynesian seafarers, including Tongans, Samoans, Niueans, Cook Islanders, Tahitians, Hawaiians, Marquesans and Māori, are genetically linked to indigenous peoples of parts of Southeast Asia, including those of Taiwan.
demonstrated that this Sicilian taxon has a distinct mitochondrial haplotype differing in this respect from all other circum-Mediterranean green toad species.
He was also an NIH fellow in the laboratory of Peter Mitchell at Glynn Research Laboratories in Bodmin, England studying mitochondrial amino acid transport.
Related family members include phage T3 and SP6 RNA polymerases, but this family is also related to the mitochondrial RNA polymerase.
Translocase of outer mitochondrial membrane 40 (TOMM40), a protein encoded by the TOMM40 gene, whose alleles differentially impact the risk for Alzheimer's disease