acyl-CoA dehydrogenase

acyl-CoA dehydrogenase

/ac·yl-CoA de·hy·dro·gen·ase/ (de-hi´dro-jen-ās) any of several enzymes that catalyze the oxidation of acyl coenzyme A thioesters as a step in the degradation of fatty acids. Individual enzymes are specific for certain ranges of acyl chain lengths: long-chain a.-CoA d. (LCAD), medium-chain a.-CoA d. (MCAD), and short-chain a.-CoA d. (SCAD).
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Background: Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency (MADD) from immune-mediated necrotizing myopathy (IMNM) because they display similar symptoms.
Additionally, acyl-CoA dehydrogenase, very long chain, which is involved in a fatty acid [beta]-oxidation pathway, was found in the QTL interval on SSC12 [14].
As Max discovers, he has medium chain acyl-CoA dehydrogenase deficiency, or MCADD.
This infant has an autosomal recessive disorder described as multiple acyl-CoA dehydrogenase deficiency (MADD).
2]) upon reduction with its substrate, medium chain acyl-CoA dehydrogenase (MCAD).
IN VITRO STUDY OF VERY LONG CHAIN ACYL-COA DEHYDROGENASE DEFICIENCY WITH MUSCULAR INVOLVEMENT
Conditions currently screened for in Wales are congenital hypothyroidism, cystic fibrosis, medium chain acyl-CoA dehydrogenase deficiency (MCADD), phenylketonuria and sickle cell disorders.
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (MIM 201475) was first described in 1993.
MACD (medium-chain acyl-CoA dehydrogenase deficiency)
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.

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