نتایج جستجو برای: mitochondrial disorders

تعداد نتایج: 792831  

Journal: :Journal of Inherited Metabolic Disease 2010

Journal: :International Journal of Molecular Sciences 2021

Journal: :The Lancet. Neurology 2010
Robert McFarland Robert W Taylor Douglass M Turnbull

Disruption of the most fundamental cellular energy process, the mitochondrial respiratory chain, results in a diverse and variable group of multisystem disorders known collectively as mitochondrial disease. The frequent involvement of the brain, nerves, and muscles, often in the same patient, places neurologists at the forefront of the interesting and challenging process of diagnosing and carin...

Journal: :Behavioural brain research 2005
Haim Einat Peixiong Yuan Husseini K Manji

There is growing evidence that anxiety disorders are associated with impairments of cellular plasticity and resilience. Paralleling these advances in our understanding of the neurobiologic underpinnings of anxiety disorders is the growing appreciation of the diverse functions that mitochondria play in regulating integrated CNS function. The emerging data suggest that mitochondrial Ca2+ sequestr...

2013
Michela Ranieri Simona Brajkovic Giulietta Riboldi Dario Ronchi Federica Rizzo Nereo Bresolin Stefania Corti Giacomo P. Comi

Mitochondria are highly dynamic, complex organelles that continuously alter their shape, ranging between two opposite processes, fission and fusion, in response to several stimuli and the metabolic demands of the cell. Alterations in mitochondrial dynamics due to mutations in proteins involved in the fusion-fission machinery represent an important pathogenic mechanism of human diseases. The mos...

2017
N. Z. Lax G. S. Gorman D. M. Turnbull

Mitochondrial respiratory chain defects are an important cause of inherited disorders affecting approximately 1 in 5000 people in the UK population. Collectively these disorders are termed 'mitochondrial diseases' and they result from either mitochondrial DNA mutations or defects in nuclear DNA. Although they are frequently multisystem disorders, neurological deficits are particularly common, w...

Journal: :Neurology 2013
Daniel S Lieber Sarah E Calvo Kristy Shanahan Nancy G Slate Shangtao Liu Steven G Hershman Nina B Gold Brad A Chapman David R Thorburn Gerard T Berry Jeremy D Schmahmann Mark L Borowsky David M Mueller Katherine B Sims Vamsi K Mootha

OBJECTIVE To evaluate the utility of targeted exome sequencing for the molecular diagnosis of mitochondrial disorders, which exhibit marked phenotypic and genetic heterogeneity. METHODS We considered a diverse set of 102 patients with suspected mitochondrial disorders based on clinical, biochemical, and/or molecular findings, and whose disease ranged from mild to severe, with varying age at o...

Journal: :CMAJ open 2013
Rebecca E Anglin Patricia I Rosebush Michael D Noseworthy Mark Tarnopolsky Alexander M Weber Noam Soreni Michael F Mazurek

BACKGROUND Mitochondrial disorders are clinical syndromes associated with mutations in the mitochondrial or nuclear genome that result in impaired oxidative phosphorylation and deficient energy production. Metabolic abnormalities in brain areas associated with cognitive functions could give rise to neuropsychiatric symptomatology. The aim of this study was to use single-voxel proton magnetic re...

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