Patients with multiple acyl-CoA
Some of the defective pathways in multiple acyl-CoA
Rat long-chain acyl-CoA
synthetase mRNA, protein, and activity vary in tissue distribution and in response to diet.
Infants suspected to have very-long chain acyl-CoA
dehydrogenase deficiency from newborn screening.
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA
Dehydrogenase deficiency Combined analysis by structural, functional and pharmacological approaches.
Very long chain acyl-CoA
dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts.
Lack of carnitine leads to toxic accumulation of long-chain fatty acids in cytoplasm and acyl-coA
In fact, it is found in every cell of the body, where it is an intracellular transporter of a metabolite called acyl-CoA
We investigated whether the age-related impairment of cardiac fatty acid catabolism occurs, at least partially, through diminished levels of L-carnitine, which would adversely affect carnitine palmitoyltransferase 1 (CPT1), the rate-limiting enzyme for fatty acyl-CoA
uptake into mitochondria for beta]-oxidation.
MACD (medium-chain acyl-CoA
We were able to save about 15 babies with medium-chain acyl-CoA
dehydrogenase or MCAD deficiency, a condition that prevents the body from converting certain fats in food to energy," Dr al-Rifai said, adding: "The early detection has allowed for early treatment.
Primer sequences for acetyl-CoA acyltransferase (ACAA2), long-chain acyl-CoA
dehydrogenase (ACADL), acyl-CoA
synthetase (ACSL1), very long chain acyl-CoA
dehydrogenase (ACADVL), carnitine palmitoyltransferase 1B (CPT1B), enoyl-CoA hydratase (ECH1), hydroxyacyl-CoA dehydrogenase (HADHA), pyruvate dehydrogenase kinase (PDK4), PGC-1[alpha], PGC-1[beta], PPAR[alpha], glutamate dehydrogenase, and 18S ribosomal RNA are available in Supplemental Material, Table S1 (http://dx.