نتایج جستجو برای: mitochondrial mutations

تعداد نتایج: 298675  

2017
Nuno Rocha David A Bulger Andrea Frontini Hannah Titheradge Sigrid Bjerge Gribsholt Rachel Knox Matthew Page Julie Harris Felicity Payne Claire Adams Alison Sleigh John Crawford Anette Prior Gjesing Jette Bork-Jensen Oluf Pedersen Inês Barroso Torben Hansen Helen Cox Mary Reilly Alex Rossor Rebecca J Brown Simeon I Taylor Duncan McHale Martin Armstrong Elif A Oral Vladimir Saudek Stephen O'Rahilly Eamonn R Maher Bjørn Richelsen David B Savage Robert K Semple

MFN2 encodes mitofusin 2, a membrane-bound mediator of mitochondrial membrane fusion and inter-organelle communication. MFN2 mutations cause axonal neuropathy, with associated lipodystrophy only occasionally noted, however homozygosity for the p.Arg707Trp mutation was recently associated with upper body adipose overgrowth. We describe similar massive adipose overgrowth with suppressed leptin ex...

Journal: :The Journal of biological chemistry 2013
Patrick S Ward Chao Lu Justin R Cross Omar Abdel-Wahab Ross L Levine Gary K Schwartz Craig B Thompson

Monoallelic point mutations in cytosolic isocitrate dehydrogenase 1 (IDH1) and its mitochondrial homolog IDH2 can lead to elevated levels of 2-hydroxyglutarate (2HG) in multiple cancers. Here we report that cellular 2HG production from cytosolic IDH1 mutation is dependent on the activity of a retained wild-type IDH1 allele. In contrast, expression of mitochondrial IDH2 mutations led to robust 2...

Journal: :The Journal of Cell Biology 2000
Peter Fekkes Kelly A. Shepard Michael P. Yaffe

Mitochondrial morphology and function depend on MGM1, a Saccharomyces cerevisiae gene encoding a dynamin-like protein of the mitochondrial outer membrane. Here, we show that mitochondrial fragmentation and mitochondrial genome loss caused by lesions in MGM1 are suppressed by three novel mutations, gag1, gag2, and gag3 (for glycerol-adapted growth). Cells with any of the gag mutations displayed ...

2016
Amy E. Vincent Hannah S. Rosa Charlotte L. Alston John P. Grady Karolina A. Rygiel Mariana C. Rocha Rita Barresi Robert W. Taylor Doug M. Turnbull

Dysferlinopathies are caused by mutations in the DYSF gene and patients may present with proximal or distal myopathy. Dysferlin is responsible for membrane resealing, and mutations may result in a defect in membrane repair following mechanical or chemical stress, causing an influx of Ca2+. Since mitochondria are involved in Ca2+ buffering, we hypothesised that mitochondrial defects may be prese...

Journal: :Mitochondrion 2008
Tomohiko Kubo Kathleen J Newton

Flowering plants harbor the largest mitochondrial genomes reported so far. At present, the nucleotide sequences of 15 mitochondrial genomes from seven angiosperm species are available, making detailed comparative analysis feasible. The gene content is variable among the species, but the most striking feature is the fluidity of intergenic regions, where species-specific sequences predominate. Ad...

2017
Nicoletta Plotegher Michael R. Duchen

Parkinson's disease (PD) is the most common motor neurodegenerative disorder. In most cases the cause of the disease is unknown, while in about 10% of subjects, it is associated with mutations in a number of different genes. Several different mutations in 15 genes have been identified as causing familial forms of the disease, while many others have been identified as risk factors. A striking nu...

Journal: :The Journal of Cell Biology 1985
B R Oakley J E Rinehart

We have examined the effects of the antimicrotubule agent benomyl and several mutations on nuclear and mitochondrial movement in germlings of the filamentous fungus Aspergillus nidulans. While, as previously reported, benomyl inhibited nuclear division and movement, it did not inhibit mitochondrial movement. To test the effects of benomyl more rigorously, we germinated two benomyl super-sensiti...

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