VLCFAVery Long-Chained Fatty Acid
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The first step of VLCFA synthesis is condensation, which is carried out by one of ELOVL1-7, each with different FA substrate preferences.
Dietary restriction of VLCFA and Lorenzo's oil were not applied in our patient because of noncompliance from his family.
It is associated with a deficiency of lignoceroyl-coenzyme A ligase, a peroxisomal enzyme needed for the oxidation of saturated very-long-chain fatty acids (VLCFAs).
Currently, measurement of the peroxisomal fatty acid [beta]-oxidation activity is performed with 1-[[sup.14]C]-radio-labeled VLCFA substrates and one of two available methods: either in intact human skin fibroblasts cultured in monolayer (5); or in isolated fibroblasts permeabilized with digitonin (6).
In an earlier report (3), we indicated that the posterior vacuole had properties of peroxisomes, which function primarily to process very long chain fatty acids (VLCFA) with the assistance of key enzymes, acyl-CoA oxidase (ACOX) and catalase.
Adrenoleukodystrophy is a rapid, progressive demyelinating disease affecting the CNS that is characterized by large increases in plasma and tissue very long saturated fatty acids (VLCFA).
thaliana fatty acid elongase gene (FAE1; encoding the seed-specific-elongase condensing enzyme) in Arabidopsis, tobacco (Nicotiana tabacum L.), and yeast, Miller and Kunst (1997) proposed that of the four enzymes involved in VLCFA biosynthesis, the two reductases and the dehydrase are expressed throughout the plant and are common to all microsomal FAE systems.
Hugo Moser of the Kennedy Krieger Institute in Baltimore, it has brought the VLCFA level of nearly all of them under control.
Abnormal plasma samples were those leftover from patients with peroxisomal diseases referred to our laboratory and that gave abnormal VLCFA results.
The 25-week proof-of-concept study, conducted at the Kennedy Krieger Institute under a sponsored research agreement with Viking, was designed to evaluate changes in VLCFA levels in the ABCD1 knockout mouse model intended to mirror the loss of ABCD1 transporter activity that is considered the hallmark of X-ALD.