The first step of the [beta]-oxidation cycle is catalyzed by acyl-coenzyme A dehydrogenases and acyl-CoA
oxidases in the two respective systems.
4] Human genes: GALC, galactosylceramidase; ACADM, acyl-CoA
dehydrogenase, C-4 to C-12 straight chain; ACADVL, acyl-CoA
dehydrogenase, very long chain; GALT, galactose-1-phosphate uridyl transferase; PAH, phenylalanine hydroxylase; FCGR2A, Fc fragment of IgG, low affinity IIa, receptor (CD32); RPP30, ribonuclease P/MRP 30kDa subunit.
Conversely, ACSL3, which encodes an acyl-CoA
synthetase enzyme involved in FA biosynthesis, was down-regulated.
Carnitine is an amino acid derivative whose primary roles in the human body are in transporting long-chain fatty acids into the mitochondria for use as a fuel and buffering excess acyl-CoA
accumulation within mitochondria.
Based on both dietary and in vitro studies of lipid metabolism, we hypothesized that the 2 isomers of CLA may have differing effects on acyl-CoA
oxidase (ACO), 3-hydroxy, 3-methylglutaryl CoA reductase (HMG-R), and apolipoprotein A-I(Apo A-I) gene expression.
An international team of doctors recently reported successes in using ketones to treat three children with the rare genetic disease known as multiple acyl-CoA
dehydrogenase deficiency, or MADD.
Another is MCAD (medium chain acyl-CoA
dehydrogenase deficiency), a disorder that affects the ability to process fat and may be responsible for up to 10 percent of deaths attributed to sudden infant death syndrome.
Calgene LLC (Davis, CA) has patented nucleic acid sequences encoding for acyl-CoA
:cholesterol acyltransferase (ACAT) related proteins, wherein ACAT-like protein is active in the formation of a sterol ester and/or triacylylgycerol from a fatty acyl-CoA
and sterol and/or diacylglycerol substrates.
In this study, DR did not affect the expression of several genes in the LM for lipid metabolism (adipose triglyceride lipase, acyl-CoA
synthetase long-chain family member, glycerol-3-phosphate acyltransferase, and hydroxyacyl-Coenzyme A dehydrogenase) and fatty acid uptake (fatty acid translocase and lipoprotein lipase) at both P1 and P2.
Rapid, comprehensive screening of the human medium chain acyl-CoA
Those babies who have their newborn bloodspot sample from this month will be offered screening for medium chain acyl-CoA
dehydrogenase deficiency (MCADD).
In another inherited disorder, medium-chain acyl-CoA
dehydrogenase deficiency (MCAD), the enzyme that is responsible for breaking down fats into energy, is missing or does not work correctly.