Carnitine palmitoyltransferase II deficiency is an autosomal recessively inherited genetic metabolic disorder characterized by an enzymatic defect that prevents long-chain fatty acids from being transported into the mitochondria for utilization as an energy source. The disorder presents in one of … See more The three main types of carnitine palmitoyltransferase II deficiency classified on the basis of tissue-specific symptomatology and age of onset. Among the few people diagnosed with CPT2, some have … See more Carnitine is a hydrophilic natural substance acquired mostly through dietary meats and dairy products and is used by cells to transport hydrophobic fatty acids. The "carnitine shuttle" is … See more Standard of care for treatment of CPT II deficiency commonly involves limitations on prolonged strenuous activity and the following stipulations: • The medium-chain fatty acid triheptanoin appears to be an effective therapy for adult-onset CPT II … See more This article incorporates public domain text from The U.S. National Library of Medicine • GeneReviews/NCBI/NIH/UW entry on Carnitine Palmitoyltransferase II Deficiency See more Enzyme structure The CPT system directly acts on the transfer of fatty acids between the cytosol and the inner mitochondrial matrix. CPT II shares structural … See more • Tandem mass spectrometry: non-invasive, rapid method; a significant peak at C16 is indicative of generalized CPT II deficiency See more • Carnitine O-palmitoyltransferase • Carnitine palmitoyltransferase I deficiency • Fasciculation See more WebCPD2 LED Curve Pendant – Direct with Flush Snap-in Lens Job Name Fixture Type Rev.0621 Page 1 Standard Arcs Available in all Radii including Full Circle LED direct curve pendants easily join with our linear direct pendants to create virtually any illuminated shape or pattern. Curve pendants utilize high performance LED boards and flush snap ...
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