Disease relevance of hexacosanoic acid

• Adrenoleukodystrophy (ALD) is an X-linked disorder characterized by demyelination, adrenal insufficiency, and accumulation of saturated very-long-chain fatty acids (VLFA), particularly hexacosanoate (C26:0) [1].
• The presence of a peroxisomal disorder was demonstrated by a greatly increased level of phytanic acid and slightly increased levels of hexacosanoate in serum and by reduced activity of peroxisomal dihydroxyacetone phosphate acyltransferase and a slightly increased ratio of cytosolic to peroxisomal catalase activity in cultured fibroblasts [2].

High impact information on hexacosanoic acid

• Competition experiments indicate that in fibroblasts, as in rat liver, distinct enzyme systems are responsible for the oxidation of palmitate on the one hand and lignocerate and cerotate on the other hand [3].
• In fibroblasts from patients with X-linked adrenoleukodystrophy the peroxisomal oxidation of lignocerate and cerotate is impaired, but not that of palmitate [3].
• Fractionation studies indicate that in rat liver activation of cerotate and lignocerate to cerotoyl-CoA and lignoceroyl-CoA, respectively, occurs in two subcellular fractions, the endoplasmic reticulum and the peroxisomes but not in the mitochondria [3].
• Correlations of elevated levels of hexacosanoate in erythrocyte membranes with risk factors for atherosclerosis [4].
• This finding provides an explanation for the inhibitory effect of fenoprofen on lignocerate and cerotate beta-oxidation in hepatocytes [5].

Anatomical context of hexacosanoic acid

• We have investigated the contribution of peroxisomes and mitochondria to the beta-oxidation of palmitate (C16:0) and cerotate (C26:0) in intact human skin fibroblasts [6].

Associations of hexacosanoic acid with other chemical compounds

• Five new lupeol esters, lup-20(29)-ene-3beta-yl eicosanoate, docosanoate, tetracosanoate, hexacosanoate and octacosanoate, were isolated as a mixture from the twig bark of Japanese pear (Pyrus serotina Rehd.) cv. Shinko, together with lupeol and epifriedelinol [7].
• No deficiency of the crude mitochrondrial (pH 4.2), the microsomal (pH 6.0), or the myelin-localized cholesterol ester hydrolases was detected, although the activities of the myelin-localized cholesterol ester hydrolase against cholesteryl lignocerate and cerotate were too low for reliable assays [8].

References