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Carnitine palmitoyl transferase 2 deficiency

Known as: CPT-II, CPT II Deficiency, Carnitine Palmitoyltransferase II Deficiency 
A rare, autosomal recessive inherited disorder of long-chain fatty-acid oxidation caused by mutations in the CPT2 gene. The disease includes three… 
National Institutes of Health

Related topics

Related topics

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Broader (2)

Carnitine O-PalmitoyltransferaseInborn Errors of Metabolism
Carnitine Palmitoyltransferase IIConditions tested for in this newborn screening study:ID:Pt:Bld.dot:NomFatty acid oxidation conditions suspected:Prid:Pt:Bld.dot:NomNewborn conditions with equivocal markers:Prid:Pt:Bld.dot:Nom

Papers overview

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Highly Cited
2011
Highly Cited
2011
Oxidation of Hepatic Carnitine Palmitoyl Transferase-I (CPT-I) Impairs Fatty Acid Beta-Oxidation in Rats Fed a Methionine-Choline Deficient Diet
There is growing evidence that mitochondrial dysfunction, and more specifically fatty acid β-oxidation impairment, is involved in… 
Highly Cited
2005
Highly Cited
2005
Malonyl-CoA decarboxylase inhibition suppresses fatty acid oxidation and reduces lactate production during demand-induced ischemia.
The rate of cardiac fatty acid oxidation is regulated by the activity of carnitine palmitoyltransferase-I (CPT-I), which is… 
Highly Cited
2005
Highly Cited
2005
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations
Patients with the myopathic form of carnitine palmitoyltransferase II (CPT II) deficiency typically experience muscle pain… 
2003
2003
Differential effects of peroxisome proliferator-activated receptor activators on the mRNA levels of genes involved in lipid metabolism in primary human monocyte-derived macrophages.
Peroxisome proliferator-activated receptors (PPARs) are key regulators of macrophage lipid metabolism. We compared the effects of… 
Highly Cited
2001
Highly Cited
2001
Long-chain fatty acids regulate liver carnitine palmitoyltransferase I gene (L-CPT I) expression through a peroxisome-proliferator-activated receptor alpha (PPARalpha)-independent pathway.
Liver carnitine palmitoyltransferase I (L-CPT I) catalyses the transfer of long-chain fatty acid (LCFA) for translocation across… 
2000
2000
Identification of Positive and Negative Determinants of Malonyl-CoA Sensitivity and Carnitine Affinity within the Amino Termini of Rat Liver- and Muscle-type Carnitine Palmitoyltransferase I*
The extreme amino terminus and, in particular, residue Glu-3 in rat liver (L) carnitine palmitoyltransferase I (CPT I) have… 
Highly Cited
2000
Highly Cited
2000
Effects of conjugated linoleic acid isomers on lipid-metabolizing enzymes in male rats
Male weanling Wistar rats (n=15), weighing 200–220 g, were allocated for 6 wk to diets containing 1% (by weight) of conjugated… 
Highly Cited
1998
Highly Cited
1998
Carnitine palmitoyltransferase I, carnitine palmitoyltransferase II, and Acyl-CoA oxidase activities in atlantic salmon (Salmo salar)
Salmon farmers are currently using high-energy feeds containing up to 35% fat; the fish's capability of fully utilizing these… 
Highly Cited
1997
Highly Cited
1997
Control of hepatic fatty acid oxidation by 5'-AMP-activated protein kinase involves a malonyl-CoA-dependent and a malonyl-CoA-independent mechanism.
Incubation of rat hepatocytes with 5-aminoimidazole-4-carboxamide ribonucleoside (AICAR), an activator of the 5'-AMP-activated… 
Review
1997
Review
1997
Thia fatty acids, metabolism and metabolic effects.
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