نتایج جستجو برای: mitochondrial myopathy

تعداد نتایج: 143464  

2017
René G. Feichtinger Monika Oláhová Yoshihito Kishita Caterina Garone Laura S. Kremer Mikako Yagi Takeshi Uchiumi Alexis A. Jourdain Kyle Thompson Aaron R. D’Souza Robert Kopajtich Charlotte L. Alston Johannes Koch Wolfgang Sperl Elisa Mastantuono Tim M. Strom Saskia B. Wortmann Thomas Meitinger Germaine Pierre Patrick F. Chinnery Zofia M. Chrzanowska-Lightowlers Robert N. Lightowlers Salvatore DiMauro Sarah E. Calvo Vamsi K. Mootha Maurizio Moggio Monica Sciacco Giacomo P. Comi Dario Ronchi Kei Murayama Akira Ohtake Pedro Rebelo-Guiomar Masakazu Kohda Dongchon Kang Johannes A. Mayr Robert W. Taylor Yasushi Okazaki Michal Minczuk Holger Prokisch

Complement component 1 Q subcomponent-binding protein (C1QBP; also known as p32) is a multi-compartmental protein whose precise function remains unknown. It is an evolutionary conserved multifunctional protein localized primarily in the mitochondrial matrix and has roles in inflammation and infection processes, mitochondrial ribosome biogenesis, and regulation of apoptosis and nuclear transcrip...

Journal: :Archives of neurology 1998
K G Kimata L Gordan E T Ajax P H Davis T Grabowski

We describe a 60-year-old man with MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes) and discuss the mitochondrial DNA point mutation 3243. A diagnosis of MELAS should be considered in the appropriate clinical setting at any age.

2013
Vesna Stojanović Johannes A. Mayr Wolfgang Sperl Nenad Barišić Aleksandra Doronjski Gordana Milak

Mitochondrial DNA depletion syndromes are a group of autosomal recessive hereditary disorders characterized by reduction of the amount of mitochondrial DNA in the affected tissue (muscle, liver, brain, or kidneys). We report a case of an infant with myopathy, deafness, peripheral neuropathy, nephrocalcinosis, proximal renal tubulopathy, moderate lactic acidosis, and a novel mutation of the RRM2...

Journal: :Human molecular genetics 2010
Sofia Ahola-Erkkilä Christopher J Carroll Katja Peltola-Mjösund Valtteri Tulkki Ismo Mattila Tuulikki Seppänen-Laakso Matej Oresic Henna Tyynismaa Anu Suomalainen

Mitochondrial dysfunction is a major cause of neurodegenerative and neuromuscular diseases of adult age and of multisystem disorders of childhood. However, no effective treatment exists for these progressive disorders. Cell culture studies suggested that ketogenic diet (KD), with low glucose and high fat content, could select against cells or mitochondria with mutant mitochondrial DNA (mtDNA), ...

2012
Gloria P. Duran H. Poggi

Background: Mitochondrial diseases are a group of disorders caused by mutations in nuclear DNA or mitochondrial DNA, usually involving multiple organ systems. Primary adrenal insufficiency due to mitochondrial disease is extremely infrequent and has been reported in association with mitochondrial DNA deletion syndromes such as Kearns–Sayre syndrome. Aim: To report a 3-year-old boy with Addison ...

Journal: :Neurology India 2004
Sundaram Challa Meena A Kanikannan Jagarlapudi Mk Murthy Venkateswar R Bhoompally Mohandas Surath

BACKGROUND Mitochondrial diseases are caused by mutations in mitochondrial or nuclear genes, or both and most patients do not present with easily recognizable disorders. The characteristic morphologic change in muscle biopsy, ragged-red fibers (RRFs) provides an important clue to the diagnosis. MATERIALS AND METHODS Demographic data, presenting symptoms, neurological features, and investigati...

2017
Yu-Xing Ge Bo Shang Wen-Zhen Chen You Lu Jue Wang

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a clinical syndrome associated with mitochondrial disorders (MIDs). This report illustrates a case of MELAS syndrome with hypothyroidism and psychiatric disorders, which is different from the common clinical manifestations of MELAS syndrome, such as exercise intolerance, migraine-like headaches, hearing ...

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