acyl-CoA dehydrogenases
acyl-CoA dehydrogenases
Enzymes that activate the first stage of the oxidation of fatty acids.Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
Mentioned in
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- 2-enoyl-CoA reductase
- ACAD10
- ACAD11
- ACADL
- ACADM
- ACADS
- ACADSD
- ACADVL
- ACADVLD
- ACD
- acyl-CoA dehydrogenase short-chain deficiency
- acyl-CoA dehydrogenase type-9 deficiency
- acyl-CoA dehydrogenase very long-chain deficiency
- ceramidase deficiency
- deficiency of sweating
- dicarboxylic aciduria due to defective beta-oxidation of fatty acids
- GCDH
- glutaric acidemia type 2
- glutaryl-CoA dehydrogenase
References in periodicals archive
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The [beta]-oxidation cycle consists of four reactions, catalyzed by acyl-CoA dehydrogenases (ACADs), enoyl-CoA hydratases, L-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase, that sequentially remove two carbons--one acetyl-CoA molecule, until the acyl-CoA is completely converted to acetyl-CoA.
Tanaka, "Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria.
Selective Inhibition of Acyl-CoA Dehydrogenases by a Metabolite of Hypoglycin.
In my own specialist area of lipid metabolism, deficiencies of the fatty acid metabolizing acyl-CoA dehydrogenases are defined as a group of diseases that may be potentially treatable with a combination of dietary manipulation and prevention of fasting, because these are essentially diseases of fasting intolerance.
The first step of the [beta]-oxidation cycle is catalyzed by acyl-CoA dehydrogenases, whereas the enzymes of MTP catalyze the last 3 steps of [beta]-oxidation.
This is in contrast to the situation for human very long-chain, long-chain, and medium-chain acyl-CoA dehydrogenases, which are stereospecific for the S-enantiomers only of their methyl branched-chain substrates (24).
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: iden tification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism.
MADD patients showed the whole range of unusual fatty acids observed in both MCAD and vLCAD deficiencies, reflecting the generalized defect of all the mitochondrial acyl-CoA dehydrogenases. In the neonatal form, there was a strong increase of all these metabolites, whereas in the mild forms of MADD, the increase of [C.sub.10:1] and [C.sub.12:1] was pathognomonic, and the increase of [C.sub.14:2].
In special severe metabolic diseases of [beta]-oxidation, like multiple acyl-CoA dehydrogenase deficiency (MADD), a direct intake of ketone bodies for energy supply is necessary.
First described in a neonate with hypotonia who subsequently developed a severe pyramidal disorder, EE was initially labeled as a branched-chain acyl-CoA dehydrogenase defect (1).
ACADM, a member of the acyl-CoA dehydrogenase (ACAD) family that comprises 9 known proteins, is involved in the oxidation of medium-chain fatty acids and amino acids (Kim Miura, 2004).
The four enzymes making up this pathway, in the order that they are utilized, are: (a) an acyl-CoA dehydrogenase, (b) a 2,3-encyl-CoA hydratase, (c) an L-3-hydroxyacyl-CoA dehydrogenase, and (d) a 3-ketoacyl-CoA thiolase.
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- Acyl Protein Thioesterase 1
- acyl-ACP dehydrogenase
- acyl-ACP reductase
- acyl-activating enzyme
- Acyl-Carrier Protein
- Acyl-CoA
- Acyl-CoA
- Acyl-CoA
- acyl-CoA dehydrogenase (NADPH)
- acyl-CoA dehydrogenase family member 10
- acyl-CoA dehydrogenase family member 11
- Acyl-CoA dehydrogenase family member 8
- acyl-CoA dehydrogenase family member 9, mitochondrial
- acyl-CoA dehydrogenase family, member 10
- acyl-CoA dehydrogenase family, member 11
- acyl-CoA dehydrogenase family, member 8
- acyl-CoA dehydrogenase family, member 9
- acyl-CoA dehydrogenase NADPH
- acyl-CoA dehydrogenase short-chain deficiency
- acyl-CoA dehydrogenase type-9 deficiency
- acyl-CoA dehydrogenase very long-chain deficiency
- acyl-CoA dehydrogenase, C-2 to C-3 short chain
- acyl-CoA dehydrogenase, C-4 to C-12 straight chain
- acyl-CoA dehydrogenase, long chain
- Acyl-coa dehydrogenase, long-chain
- Acyl-CoA Dehydrogenase, Medium-Chain
- Acyl-CoA Dehydrogenase, Short-Chain
- Acyl-CoA Dehydrogenase, Short/Branched Chain
- acyl-CoA dehydrogenase, very long chain
- Acyl-CoA Dehydrogenase, Very Long-Chain
- acyl-CoA dehydrogenases
- acyl-CoA synthetase
- acyl-CoA synthetase short-chain family member 2
- Acyl-CoA Thioester Hydrolase
- acyl-coenzyme A
- acyl-coenzyme A
- acyl-Coenzyme A dehydrogenase family, member 10
- acyl-Coenzyme A dehydrogenase family, member 11
- acyl-Coenzyme A dehydrogenase family, member 8
- acyl-Coenzyme A dehydrogenase family, member 9
- acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain
- acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain
- acyl-Coenzyme A dehydrogenase, long chain
- acyl-Coenzyme A dehydrogenase, very long chain
- Acyl-Coenzyme A Oxidase
- Acyl-coenzyme A:cholesterol acyltransferase
- Acyl-Coenzyme A:Cholesterol O-Transferase
- Acyl-Coenzyme A:Cholesterol O-Transferase
- acyl-malonyl-ACP synthase
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- acylamidase
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- acylated plasminogen streptokinase complex
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