It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS. (bvsalud.org)
Mitochondrial1
1] Intracellular carnitine deficiency impairs the entry of long-chain fatty acids into the mitochondrial matrix. (medscape.com)
Fatty acids2
It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. (medscape.com)
Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) is also impaired. (medscape.com)
Acyl-CoA esters2
Regulation of the intramitochondrial free CoA also is affected, with accumulation of acyl-CoA esters in the mitochondria. (medscape.com)
Can promote excretion of excess fatty acids in patients with defects in fatty acid metabolism or specific organic acidopathies, which bioaccumulate acyl CoA esters. (medscape.com)
1.171
Tiene actividades enoil-CoA hidratasa (EC 4.2.1.17) y 3-hidroxiacil-CoA (de cadena larga) deshidrogenasa (EC 1.1.1.211). (bvsalud.org)
Carnitine3
Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA. (medscape.com)
Carnitine therapy for long-chain fatty acid oxidation defects has become questionable because it promotes formation of long-chain acylcarnitines that may cause arrhythmogenesis and membrane dysfunction. (medscape.com)
In secondary carnitine deficiency, carnitine enhances excretion of toxic metabolites and generation of free CoA. (medscape.com)
Dehydratase1
This cluster encodes an acyl carrier protein (denoted as "amxACP") and a variant of FabZ, an ACP-3-hydroxyacyl dehydratase. (nih.gov)
Acylcarnitines1
Long-chain acylcarnitines are also toxic and may have an arrhythmogenic effect, causing sudden cardiac death. (medscape.com)
Fatty2
It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. (medscape.com)
Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) is also impaired. (medscape.com)
Type1
It has proven useful for the treatment of some patients with SCAD deficiency, riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency, and milder forms of glutaric aciduria type II. (medscape.com)