نتایج جستجو برای: mitochondrial trifunctional protein
تعداد نتایج: 1334579 فیلتر نتایج به سال:
3,4-dihydro-3-phenyl-2H-1,3-benzoxazines derived from phenol-, resorcinol-, and phloroglucinol give monomers with one, two, and three oxazine units at a single benzene ring, respectively. Aside from the synthesis and characterization of such multifunctional benzoxazines, reactivity and polymerization behavior is studied in dependence of the oxazine functionality. Monomer reactivities are direct...
Human mitochondrial trifunctional protein (TFP) is a heterooctamer of four alpha- and four beta-subunits that catalyzes three steps in the beta-oxidation spiral of long-chain fatty acids. TFP deficiency causes a Reye-like syndrome, cardiomyopathy, or sudden, unexpected death. We delineated the molecular basis for TFP deficiency in two patients with a unique phenotype characterized by chronic pr...
A simple synthetic strategy was developed for the synthesis of well-defined multifunctional poly(amide)-based dendrimers using a trifunctional core.
BACKGROUND The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS Over a period of 25 years, 107 patients were diagnosed wi...
-The behavior near the gelation threshold of trifunctional randomly branched polycyanurates is studied by static and dynamic light scattering. By static measurements the
mitochondria play key roles in many cell functions including energy production, fatty acid metabolism, pyrimidine biosynthesis, calcium homeostasis, and aging. they also regulate crucial signaling cascades such as apoptosis and oxidative stress. the proteome is often used to investigate the functional correlations on protein levels. based upon the human, genome there is estimated 2000 to 2500 a...
Over the last years acylcarnitines have emerged as important biomarkers for the diagnosis of mitochondrial fatty acid beta-oxidation (mFAO) and branched-chain amino acid oxidation disorders assuming they reflect the potentially toxic acyl-CoA species, accumulating intramitochondrially upstream of the enzyme block. However, the origin of these intermediates still remains poorly understood. A pos...
A new trifunctional probe, assembled using a cleavable linker, is useful for efficient enrichment and detection of alkynyl sugar-tagged biomolecules.
High efficiency orange OLEDs have been achieved using a trifunctional Pt(II) complex that contains an electron-transporting triarylborane and a hole-transporting triarylamine.
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