نتایج جستجو برای: mitochondrial mutations

تعداد نتایج: 298675  

Journal: :Genetics 2002
Theodor Hanekamp Mary K Thorsness Indrani Rebbapragada Elizabeth M Fisher Corrine Seebart Monica R Darland Jennifer A Coxbill Dustin L Updike Peter E Thorsness

In the yeast Saccharomyces cerevisiae, certain mutant alleles of YME4, YME6, and MDM10 cause an increased rate of mitochondrial DNA migration to the nucleus, carbon-source-dependent alterations in mitochondrial morphology, and increased rates of mitochondrial DNA loss. While single mutants grow on media requiring mitochondrial respiration, any pairwise combination of these mutations causes a re...

Journal: :Mitochondrion 2007
Ali-Reza Moslemi Niklas Darin

Mitochondrial OXPHOS disorders are caused by mutations in mitochondrial or nuclear genes, which directly or indirectly affect mitochondrial oxidative phosphorylation (OXPHOS). Primary mtDNA abnormalities in children are due to rearrangements (deletions or duplications) and point mutations or insertions. Mutations in the nuclear-encoded polypeptide subunits of OXPHOS result in complex I and II d...

Journal: :Bioscience reports 2007
Salvatore DiMauro

The small, maternally inherited mitochondrial DNA (mtDNA) has turned out to be a hotbed of pathogenic mutations: 15 years into the era of 'mitochondrial medicine', over 150 pathogenic point mutations and countless rearrangements have been associated with a variety of multisystemic or tissue-specific human diseases. MtDNA-related disorders can be divided into two major groups: those due to mutat...

Journal: :Brain : a journal of neurology 2012
Dario Ronchi Caterina Garone Andreina Bordoni Purificacion Gutierrez Rios Sarah E Calvo Michela Ripolone Michela Ranieri Mafalda Rizzuti Luisa Villa Francesca Magri Stefania Corti Nereo Bresolin Vamsi K Mootha Maurizio Moggio Salvatore DiMauro Giacomo P Comi Monica Sciacco

The molecular diagnosis of mitochondrial disorders still remains elusive in a large proportion of patients, but advances in next generation sequencing are significantly improving our chances to detect mutations even in sporadic patients. Syndromes associated with mitochondrial DNA multiple deletions are caused by different molecular defects resulting in a wide spectrum of predominantly adult-on...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 2007
Suhail K Mithani Janis M Taube Shaoyu Zhou Ian M Smith Wayne M Koch William H Westra Joseph A Califano

PURPOSE To determine the timing of mitochondrial mutations in the progression of head and neck squamous cell carcinoma. EXPERIMENTAL DESIGN Twenty-three mitochondrial mutations were identified in 12 tumors using a high-throughput mitochondrial sequencing array. Areas of adjacent dysplastic and normal epithelium adjacent to tumors were sequenced using conventional methods for the presence of m...

2001
Chan Bae Park Nils-Göran Larsson

The human mitochondrial genome involves over 1,000 genes, dispersed across the maternally inherited mitochondrial DNA (mtDNA) and the biparentally inherited nuclear DNA (nDNA). The mtDNA encodes 13 core proteins that determine the efficiency of the mitochondrial energy-generating system, oxidative phosphorylation (OXPHOS), plus the RNA genes for their translation within the mitochondrion. The m...

2016
Steven A. Hardy Emma L. Blakely Andrew I. Purvis Mariana C. Rocha Syeda Ahmed Gavin Falkous Joanna Poulton Michael R. Rose Olivia O'Mahony Niamh Bermingham Charlotte F. Dougan Yi Shiau Ng Rita Horvath Doug M. Turnbull Grainne S. Gorman Robert W. Taylor

Pathogenic mitochondrial tRNA (mt-tRNA) gene mutations represent a prominent cause of primary mitochondrial DNA (mtDNA)-related disease despite accounting for only 5%-10% of the mitochondrial genome.(1,2) Although some common mt-tRNA mutations, such as the m.3243A>G mutation, exist, the majority are rare and have been reported in only a small number of cases.(3) The MT-TP gene, encoding mt-tRNA...

2013
Igor A. Sobenin Margarita A. Sazonova Anton Y. Postnov Jukka T. Salonen Yuri V. Bobryshev Alexander N. Orekhov

In human pathology, several diseases are associated with somatic mutations in the mitochondrial genome (mtDNA). Even though mitochondrial dysfunction leads to increased oxidative stress, the role of mitochondrial mutations in atherosclerosis has not received much attention so far. In this study we analyzed the association of mitochondrial genetic variation with the severity of carotid atheroscl...

Journal: :Nature reviews. Genetics 2001
S DiMauro E A Schon

The small, maternally inherited mitochondrial DNA (mtDNA) has turned out to be a Pandora's box of pathogenic mutations: 13 years into the era of "molecular mitochondrial medicine," more than 100 pathogenic point mutations and innumerable rearrangements have been associated with a striking variety of multisystemic as well as tissue-specific human diseases. After reviewing the principles of mitoc...

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