نتایج جستجو برای: neurometabolic disorders

تعداد نتایج: 671446  

Journal: :Journal of Bioenergetics and Biomembranes 2015

Journal: :Brain research. Brain research reviews 2005
Jon T Sakata David Crews F Gonzalez-Lima

Cytochrome oxidase is a rate-limiting enzyme in oxidative phosphorylation, the major energy-synthesizing pathway used by the central nervous system, and cytochrome oxidase histochemistry has been extensively utilized to map changes in neural metabolism following experimental manipulations. However, the value of cytochrome oxidase activity in predicting behavior has not been analyzed. We argue t...

2015
Sayena JABBEHDARI Elham RAHIMIAN Narjes JAFARI Sara SANII Simin KHAYATZADEHKAKHKI Habibe NEJAD BIGLARI

OBJECTIVE Metachromatic leukodystrophy disorder (MLD) is one of the rare neurometabolic diseases caused due to lack of saposin B and arylsulfatase A enzyme deficiency. MATERIALS & METHODS Eighteen patients diagnosed as metachromatic leukodystrophy in the Neurology Department of Mofid Children's Hospital in Tehran, Iran between 2010 and 2014 were included in our study. The disorder was confirm...

Journal: :Human molecular genetics 2013
Chi-un Choe Christine Nabuurs Malte C Stockebrand Axel Neu Patricia Nunes Fabio Morellini Kathrin Sauter Stefan Schillemeit Irm Hermans-Borgmeyer Bart Marescau Arend Heerschap Dirk Isbrandt

Phosphorylated creatine (Cr) serves as an energy buffer for ATP replenishment in organs with highly fluctuating energy demand. The central role of Cr in the brain and muscle is emphasized by severe neurometabolic disorders caused by Cr deficiency. Common symptoms of inborn errors of creatine synthesis or distribution include mental retardation and muscular weakness. Human mutations in l-arginin...

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