نتایج جستجو برای: mitochondrial trifunctional protein
تعداد نتایج: 1334579 فیلتر نتایج به سال:
The synthesis and structural, photophysical, electrochemical, and electroluminescent properties of a novel class of trifunctional Pt(II) cyclometalated complexes are reported in which the hole-transporting triarylamine, electron-transporting oxadiazole, and electroluminescent metal components are integrated into a single molecule. These neutral metal chelates display good thermal stability (>25...
Case presentation: Female, 4 years old, hospitalized for coughing and reduced level of consciousness. Physical examination revealed tachycardia, no response to stimuli, isochoric photoreactive pupils. Diagnostic hypotheses viral encephalitis myocarditis were raised after laboratory tests did not suggest sepsis. During admission the Intensive Care Unit, skull computed tomography cerebrospinal fl...
We recently reported an autosomal dominant form of renal Fanconi syndrome caused by a missense mutation in the third codon of the peroxisomal protein EHHADH. The mutation mistargets EHHADH to mitochondria, thereby impairing mitochondrial energy production and, consequently, reabsorption of electrolytes and low-molecular-weight nutrients in the proximal tubule. Here, we further elucidate the mol...
BACKGROUND The human mitochondrial trifunctional protein (MTP) complex is composed of 4 hydroacyl-CoA dehydrogenase-alpha (HADHA) and 4 hydroacyl-CoA dehydrogenase-beta (HADHB) subunits, which catalyze the last 3 steps in the fatty acid beta-oxidation spiral of long-chain fatty acids. The HADHB gene encodes long-chain ketoacyl-CoA thiolase (LCTH) activity, whereas the HADHA gene contains the in...
Regulation of mitochondrial trifunctional protein modulates nonalcoholic fatty liver disease in mice
AIMS Cardiomyopathy is a major complication of diabetes. Our study was aimed to identify the sites of mitochondrial dysfunction and delineate its consequences on mitochondrial metabolism in a model of type 1 diabetes. METHODS AND RESULTS Diabetes was induced by streptozotocin injection to male Lewis rats. We found a decrease in mitochondrial biogenesis pathway and electron transport chain com...
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