VLCFA

Acronym	Definition
VLCFA	Very 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.

Rapamycin inhibits expression of elongation of very-long-chain fatty acids 1 and synthesis of docosahexaenoic acid in bovine mammary epithelial cells

Kranial MR bulgusu olan bir hastada klinik bulgu olmasa da ALD suphesini dislamak icin serum VLCFA duzeyleri degerlendirilmelidir.

Difficulties with diagnosis and differential diagnosis in childhood cerebral phenotype of adrenoleukodystrophy: a case report/Cocukluk cagi serebral tip adrenolokodistrofi'de tani ve ayirici tani zorluklari: bir olgu sunumu

Dietary restriction of VLCFA and Lorenzo's oil were not applied in our patient because of noncompliance from his family.

Uncommon cause of acute adrenal failure/Akut surrenal yetersizliginin seyrek rastlanan bir nedeni

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).

Newborn screening for X-linked adrenoleukodystrophy on the horizon

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).

Method for measurement of peroxisomal very-long-chain fatty acid ([beta]-oxidation in human skin fibroblasts using stable-isotope-labeled tetracosanoic acid

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.

Catalase in microsporidian spores before and during discharge

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).

Lorenzo's Oil and the blood brain barrier: ramifications for the treatment of x-linked Adrenoleukodystrophy

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.

Improving Erucic Acid Content in Rapeseed through Biotechnology: What Can the Arabidopsis FAE1 and the Yeast SLC1-1 Genes Contribute?(1,2)

Hugo Moser of the Kennedy Krieger Institute in Baltimore, it has brought the VLCFA level of nearly all of them under control.

Lorenzo's oil - more than just a great movie

Abnormal plasma samples were those leftover from patients with peroxisomal diseases referred to our laboratory and that gave abnormal VLCFA results.

Determination of L-pipecolic acid in plasma using chiral liquid chromatography--electrospray tandem mass spectrometry

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.

Viking Therapeutics Study of VK0214 in Mouse Model of X-ALD Demonstrates Significant Reductions in Plasma and Tissue VLCFAs