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Carnitine Palmitoyltransferase Deficiencies

Molecular Genetics and Metabolism, 1999
Carnitine palmitoyltransferase (CPT) deficiencies are common disorders of mitochondrial fatty acid oxidation. The CPT system is made up of two separate proteins located in the outer- (CPT1) and inner- (CPT2) mitochondrial membranes. While CPT2 is a ubiquitous protein, two tissue-specific CPT1 isoforms-the so-called "liver" (L) and "muscle" (M) CPT1s ...
J P, Bonnefont +6 more
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Propionyl-L-Carnitine

Drugs & Aging, 1998
Propionyl-L-carnitine stimulates energy production in ischaemic muscles by increasing citric acid cycle flux and stimulating pyruvate dehydrogenase activity. The free radical scavenging activity of the drug may also be beneficial. Propionyl-L-carnitine improves coagulative fibrinolytic homeostasis in vasal endothelium and positively affects blood ...
L R, Wiseman, R N, Brogden
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[Carnitine deficiency and carnitine therapy].

Zeitschrift fur die gesamte innere Medizin und ihre Grenzgebiete, 1988
The main function of carnitine is the transport of fatty acids across the inner mitochondrial membrane to the side of beta-oxidation. In healthy subjects no carnitine deficiency occurs. There are many inborn errors with carnitine deficiency as a primary genetic defect or secondary to other familial disorders of metabolism.
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Carnitine analogues and carnitine palmitoyltransferases

Biochemical Society Transactions, 1990
M S, Murthy, R R, Ramsay, S V, Pande
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Carnitine

1957
G, FRAENKEL, S, FRIEDMAN
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Carnitine

Annual Review of Biochemistry, 1988
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Carnitine