Titlebook: JIMD Reports, Volume 27; Eva Morava,Matthias Baumgartner,Verena Peters Book 2016 SSIEM and Springer-Verlag Berlin Heidelberg 2016 inherite

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新陳代謝

Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblastetiology, prognosis and assessing of treatment options. Here we present a cell phenotyping protocol using image cytometry that combines measurements of crucial cellular and mitochondrial parameters: (1) cell number and viability, (2) thiol redox status (TRS), (3) mitochondrial membrane potential (MM

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, Deficiency: A Deafness-Dystonia Syndrome with Distinctive Metabolic Findings (Report of a New Pat and ADP in the Krebs cycle. Disruption of . function can lead to mitochondrial DNA depletion. Patients with a . mutation present with a rare but distinctive deafness-dystonia syndrome. Additionally, they exhibit elevated levels of the characteristic biochemical markers: methylmalonate, C4-dicarboxy

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LEVER

Intracranial Pressure Monitoring Demonstrates that Cerebral Edema Is Not Correlated to Hyperammonemebral edema. However, the underlying mechanism of brain cytotoxicity remains controversial. Our objective is to present an unusual acute hyperammonemic crisis suggesting a key role of brain glutamine to mediate ammonia neurotoxicity and the interest of intracerebral pressure (ICP) monitoring to main

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Voluntary Exercise Prevents Oxidative Stress in the Brain of Phenylketonuria Mice,wn to improve brain function in hyperphenylalaninemia and neurodegenerative diseases. This study aimed to verify the effects of exercise on coordination and balance, plasma and brain amino acid levels, and brain oxidative stress markers in PKU mice...: Twenty wild-type (WT) and 20 PAH. (PKU) C57BL/6