Mitochondrial Encephalomyopathy in Drosophila

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Neurobiology of Disease Mitochondrial Encephalomyopathy in Drosophila

Alicia M. Celotto,1,2 Adam C. Frank,1,2 Steven W. McGrath,1,2 Tim Fergestad,3 Wayne A. Van Voorhies,5 Karolyn F. Buttle,4 Carmen A. Mannella,4 and Michael J. Palladino1,2 1Department of Pharmacology and 2Pittsburgh Institute for Neurodegenerative Diseases, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, 3Laboratory of Genetics, University of Wisconsin–Madison, Madis...

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Biochemical studies in mitochondrial encephalomyopathy.

The alpha-keto acid dehydrogenase complex and its component enzymes, lactate dehydrogenase, pyruvate carboxylase, cytochrome c oxidase, succinate-cytochrome c reductase, NADH-cytochrome c reductase, and the concentration of cytochromes and enzymes of beta-oxidation in muscle from a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes were studied and no ...

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mitochondrial neurogastrointestinal encephalo-myopathy (mngie) is a rare autosomal recessive disease caused by thymidine phosphorylase (tp) gene mutation. here we report a patient with mngie in whom sensorimotor polyneuropathy was the first presenting symptom and had a fluctuating course. this 26-year-old female patient developed acute-onset demyelinating polyneuropathy from the age of 6 with t...

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Non-epileptic myoclonus and mitochondrial encephalomyopathy.

Two brothers presented to us with a progressive myoclonic syndrome with slight cerebellar symptoms. Neurological examination disclosed moderate cerebellar signs and pale optic discs; asymmetric, asynchronous and arrhythmic myoclonus, an arrthesthesic deficit and no muscular weakness. EEG background activity was moderately slow with no irritative discharges. CT was normal in both cases. Intermit...

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A novel finding in MNGIE (mitochondrial neurogastrointestinal encephalomyopathy): hypergonadotropic hypogonadism.

Address for correspondence: İsmail Hakkı Kalkan, MD, Attar Sokak 21/14, Gaziosmanpaşa/ Çankaya 06700, ankara, Turkey, Tel.: +90 505 270 40 85, e-mail: [email protected] Received 13-03-12, Revised 15-06-12, Accepted 02-07-12 Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive multisystem disorder caused by mutations in the gene encoding thymidine phos...

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ژورنال

عنوان ژورنال: Journal of Neuroscience

سال: 2006

ISSN: 0270-6474,1529-2401

DOI: 10.1523/jneurosci.4162-05.2006