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Recommendations for acylcarnitine profile analysis

Annales de Biologie Clinique, 2020
Biochemical diagnosis of hereditary metabolic diseases requires the detection and simultaneous identification of a large number of compounds, hence the interest in metabolic profiles. Acylcarnitine profile allows the identification and quantification of more than thirty compounds.
Marie, Nowoczyn   +6 more
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Synthesis of dicarboxylic acylcarnitines

Chemistry and Physics of Lipids, 2004
Syntheses of malonyl, methylmalonyl, succinyl, glutaryl, methylglutaryl, dodecanedioyl and hexadecanedioyl carnitines are described. The dicarboxylic acylcarnitines were prepared from eight equivalents of cyclic anhydride or isopropylidene ester of the dicarboxylic acid and carnitine chloride in trifluoroacetic acid solution.
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Carnitine-Acylcarnitine Translocase Deficiency

The American Journal of the Medical Sciences, 1999
Carnitine-acylcarnitine translocase deficiency, like other defects of mitochondrial fatty acid oxidation, is an autosomal, recessively inherited disorder. When the deficiency is near total, it is usually fatal, affects life soon after birth, and constitutes one of the causes of skeletal muscle myopathy, cardiac and liver abnormalities, and childhood ...
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Acylcarnitine profile in thyroid disease

Clinical Biochemistry, 2013
To examine acylcarnitine profiles in individuals with hypo- or hyperthyroidism, and determine whether any atypical acylcarnitine species identified would normalize with correction of thyroid status.Serum acylcarnitine levels were measured in hypo- and hyperthyroid subjects before and after treatment with thyroxine or thionamide therapy respectively.No ...
Sophia, Wong   +5 more
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Human Forearm Arteriovenous Differences of Carnitine, Short-Chain Acylcarnitine and Long-Chain Acylcarnitine

Clinical Science, 1989
1. Forearm arterial and venous concentrations of free carnitine, short-chain acylcarnitine, long-chain acylcarnitine, glucose, lactate, pyruvate, alanine, non-esterified fatty acids, glycerol, 3-hydroxybutyrate and acetoacetate were measured in fasted adult subjects. 2. In all subjects there was net uptake of short-chain acylcarnitine, 3-
K, Bartlett   +4 more
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Acylcarnitine Deficiency in Chronic Fatigue Syndrome

Clinical Infectious Diseases, 1994
One of the characteristic complaints of patients with chronic fatigue syndrome (CFS) is the skeletal muscle-related symptom. However, the abnormalities in the skeletal muscle that explain the symptom are not clear. Herein, we show that our patients with CFS had a deficiency of serum acylcarnitine. As carnitine has an important role in energy production
H, Kuratsune   +5 more
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Acylcarnitine Analysis by Tandem Mass Spectrometry

Current Protocols in Human Genetics, 2010
AbstractCarnitine plays an essential role in fatty acid metabolism, as well as modulation of intracellular concentrations of free coenzyme A by esterification of acyl residues. Acylcarnitine analysis of various biological fluids is a sensitive method to detect >20 inborn errors of metabolism that result in abnormal accumulation of acylcarnitine ...
Emily H, Smith, Dietrich, Matern
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Properties Of Acylcarnitine Micelles.

1970
PhD ; Pharmacology ; University of Michigan, Horace H. Rackham School of Graduate Studies ; http://deepblue.lib.umich.edu/bitstream/2027.42/186160/2/7021820 ...
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Acylcarnitine hydrolase

1991
Dietmar Schomburg, Margit Salzmann
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