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A Rapid Fluorometric Method for the Determination of Carnitine Palmitoyltransferase

โœ Scribed by J. Schafer; D.M. Turnbull; H. Reichmann


Publisher
Elsevier Science
Year
1993
Tongue
English
Weight
281 KB
Volume
209
Category
Article
ISSN
0003-2697

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โœฆ Synopsis


A rapid and sensitive fluorometric assay for the measurement of carnitine palmitoyltransferase (CPT) activity is described. In this assay the coenzyme A (CoA) liberated from palmitoyl-CoA by CPT reacts with N-(9-acridinyl)-maleimide to form a fluorescent product. By comparing the fluorescence intensities of external CoA standards, CPT activity can be determined. The coefficients of variation within-run and between-run of the method were 6.9 and 10%, respectively. The CPT activity of human muscle samples measured with this assay is comparable to those obtained with a commonly used radiochemical assay and CPT deficiency in skeletal muscle from a patient was detected by this new fluorometric assay.


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Carnitine palmitoyltransferase II (CPT-II) mediates the import of long-chain fatty acids into the mitochondrial matrix for subsequent โค-oxidation. Defects of CPT-II manifest as a severe neonatal hepatocardiomuscular form or as a mild muscular phenotype in early infancy or adolescence. CPT-II deficie