نتایج جستجو برای: mitochondrial trifunctional protein

تعداد نتایج: 1334579  

2016
Marc Soto Matthias Hiller Hartmut Oschkinat Katharina Koschek

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...

Journal: :The Journal of clinical investigation 1998
J A Ibdah I Tein C Dionisi-Vici M J Bennett L IJlst B Gibson R J Wanders A W Strauss

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...

Journal: :Chemical communications 2009
Cátia Ornelas Marcus Weck

A simple synthetic strategy was developed for the synthesis of well-defined multifunctional poly(amide)-based dendrimers using a trifunctional core.

Journal: :Circulation 1999
D Bonnet D Martin Pascale De Lonlay E Villain P Jouvet D Rabier M Brivet J M Saudubray

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...

2017
Jörg Bauer Walther Burchard

-The behavior near the gelation threshold of trifunctional randomly branched polycyanurates is studied by static and dynamic light scattering. By static measurements the

Journal: :journal of paramedical sciences 0
bahram pooreydy national institute of genetic engineering and biotechnology (nigeb), tehran mohieddin jafari proteomics research center, faculty of paramedical sciences, shahid beheshti university of medical sciences, tehran faezeh tajik proteomics research center, faculty of paramedical sciences, shahid beheshti university of medical sciences, tehran mehrdad karimi traditional medicine departments, faculty of tradition medicine, tehran university of medical sciences, tehran mostafa rezaei-tavirani proteomics research center, faculty of paramedical sciences, shahid beheshti university of medical sciences, tehran alireza ghassempour department of phytochemistry, medicinal plants and drugs research institute, shahid beheshti university, tehran

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...

Journal: :Biochimica et biophysica acta 2010
Sara Violante Lodewijk Ijlst Henk van Lenthe Isabel Tavares de Almeida Ronald J Wanders Fátima V Ventura

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...

Journal: :Chemical communications 2010
Charng-Sheng Tsai Po-Yu Liu Hsin-Yung Yen Tsui-Ling Hsu Chi-Huey Wong

A new trifunctional probe, assembled using a cleavable linker, is useful for efficient enrichment and detection of alkynyl sugar-tagged biomolecules.

Journal: :Chemical communications 2011
Zachary M Hudson Michael G Helander Zheng-Hong Lu Suning Wang

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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