Genomes and Genes
Experts and Doctors on mitochondria in Clayton, Victoria, Australia
Locale: Clayton, Victoria, Australia
- Nsiah Sefaa A, McKenzie M. Combined defects in oxidative phosphorylation and fatty acid Î²-oxidation in mitochondrial disease. Biosci Rep. 2016;36: pubmed publisher..Here, we review our current understanding of the interactions between FAO and OXPHOS proteins and how defects in these two metabolic pathways contribute to mitochondrial disease pathogenesis. ..
- Myers M, Georgiou H, Byron S, Esposti M. Inhibition of mitochondrial oxidative phosphorylation induces hyper-expression of glutamic acid decarboxylase in pancreatic islet cells. Autoimmunity. 1999;30:43-51 pubmed..These results represent the first evidence that GAD expression is enhanced under conditions that are toxic to pancreatic beta cells, and establish a link between mitochondrial dysfunction and expression of IDDM autoantigens...
- Soo K, Atkin J, Farg M, Walker A, Horne M, Nagley P. Bim links ER stress and apoptosis in cells expressing mutant SOD1 associated with amyotrophic lateral sclerosis. PLoS ONE. 2012;7:e35413 pubmed publisher..These sequential links between ER stress, CHOP upregulation, and Bim activation of mitochondrial apoptotic signalling indicate a clear pathway to cell death mediated by mutant SOD1. ..
- Shingu Vazquez M, Traven A. Mitochondria and fungal pathogenesis: drug tolerance, virulence, and potential for antifungal therapy. Eukaryot Cell. 2011;10:1376-83 pubmed publisher..We discuss how the contrasting effects of mitochondrial dysfunction on fungal drug tolerance and virulence could be explained and the potential for targeting mitochondrial factors for future antifungal drug development. ..
- McKenzie M, Tucker E, Compton A, Lazarou M, George C, Thorburn D, et al. Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1. J Mol Biol. 2011;414:413-26 pubmed publisher..We conclude that C8orf38 is a crucial factor required for the translation and/or integration of ND1 into an early-stage assembly intermediate and that mutation of C8orf38 disrupts the initial stages of complex I biogenesis...
- Alcock F, Webb C, Dolezal P, Hewitt V, Shingu Vasquez M, Likic V, et al. A small Tim homohexamer in the relict mitochondrion of Cryptosporidium. Mol Biol Evol. 2012;29:113-22 pubmed publisher..We show that the atypical CpTimS exists as a relatively unstable homohexamer, shedding light both on the early evolution of the small Tim protein family and on small Tim hexamer formation in contemporary eukaryotes...
- Bursac D, Lithgow T. Jid1 is a J-protein functioning in the mitochondrial matrix, unable to directly participate in endoplasmic reticulum associated protein degradation. FEBS Lett. 2009;583:2954-8 pubmed publisher..The location of Jid1 within mitochondria makes it unavailable to participate directly in the regulation of ERAD. ..
- Lithgow T, Schneider A. Evolution of macromolecular import pathways in mitochondria, hydrogenosomes and mitosomes. Philos Trans R Soc Lond B Biol Sci. 2010;365:799-817 pubmed publisher..We discuss how the processes of protein import and tRNA import relate to each other in an evolutionary context. ..
- Ross J, McKechnie S. Micro-spatial population differentiation in activity of glycerol-3-phosphate oxidase (GPO) from mitochondria of Drosophila melanogaster. Genetica. 1991;84:145-54 pubmed..Adult GPO activity was significantly higher than that detected in larval tissues and did not show a dependence on the sugar/ethanol level in the growth medium. ..
- Clancy D. Variation in mitochondrial genotype has substantial lifespan effects which may be modulated by nuclear background. Aging Cell. 2008;7:795-804 pubmed publisher..The limited mtDNA variation in D. melanogaster makes it an ideal organism for biochemical studies to link genotype and aging phenotype. ..
- Hansen T, Smith D, Nagley P. Smac/DIABLO is not released from mitochondria during apoptotic signalling in cells deficient in cytochrome c. Cell Death Differ. 2006;13:1181-90 pubmed
- Beddoe T, Bushell S, Perugini M, Lithgow T, Mulhern T, Bottomley S, et al. A biophysical analysis of the tetratricopeptide repeat-rich mitochondrial import receptor, Tom70, reveals an elongated monomer that is inherently flexible, unstable, and unfolds via a multistate pathway. J Biol Chem. 2004;279:46448-54 pubmed..The data suggest that the malleability of the monomeric Tom70 receptor is an important factor in mitochondrial import. ..
- Bateson M, Devenish R, Nagley P, Prescott M. Single copies of subunits d, oligomycin-sensitivity conferring protein, and b are present in the Saccharomyces cerevisiae mitochondrial ATP synthase. J Biol Chem. 1999;274:7462-6 pubmed..As only the Hex6-tagged subunit was recovered from such purifications, we demonstrate that the stoichiometry of subunits d, OSCP, and b in yeast is 1 in each case. ..
- Papakonstantinou T, Law R, Nesbitt W, Nagley P, Devenish R. Molecular genetic analysis of the central hydrophobic domain of subunit 8 of yeast mitochondrial ATP synthase. Curr Genet. 1996;30:12-8 pubmed
- Finnegan P, Ellis T, Nagley P, Lukins H. The mature AEP2 gene product of Saccharomyces cerevisiae, required for the expression of subunit 9 of ATP synthase, is a 58 kDa mitochondrial protein. FEBS Lett. 1995;368:505-8 pubmed..Cell-free translation of AEP2 produces a 64 kDa polypeptide (consistent with the AEP2 sequence) which is imported into mitochondria and processed to a 58 kDa product by the removal of a presequence of about 50 amino acids. ..
- Ng H, Smith D, Nagley P. Application of flow cytometry to determine differential redistribution of cytochrome c and Smac/DIABLO from mitochondria during cell death signaling. PLoS ONE. 2012;7:e42298 pubmed publisher..Moreover, differential redistribution of cyt c and Smac occurs under various conditions, thereby reflecting constraints on availability of these proteins to exit mitochondria after permeabilization of the outer membrane. ..
- Prescott M, Bush N, Nagley P, Devenish R. Properties of yeast cells depleted of the OSCP subunit of mitochondrial ATP synthase by regulated expression of the ATP5 gene. Biochem Mol Biol Int. 1994;34:789-99 pubmed..Thus, OSCP plays a role in the assembly as well as function of the enzyme complex. ..