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Hereditary Spastic Paraparesis and Other Hereditary Myelopathies
Published in Anand D. Pandyan, Hermie J. Hermens, Bernard A. Conway, Neurological Rehabilitation, 2018
Jon Marsden, Lisa Bunn, Amanda Denton, Krishnan Padmakumari Sivaraman Nair
Adrenoleukodystrophy (ADL) is an X-linked recessive disorder characterised by adrenal insufficiency and demyelination in the central and peripheral nervous system. They are caused by a defect in a peroximal membrane transporting protein, leading to the accumulation of very-long-chain fatty acids in tissues and plasma. De novo mutations occur in 19% of cases.192 Clinical severity is not related to the length of the very-long-chain fatty acids. It occurs in 0.5–3.3 per 100,000 males and there are several forms.
Fungal Lipids
Published in Rajendra Prasad, Mahmoud A. Ghannoum, Lipids of Pathogenic Fungi, 2017
Very long-chain fatty acids (>C20) are widely distributed among the Mastigomycotina and Zygomycotina but seldom reported from Ascomycotina and Basidiomycotina. They are, however, found in mycelium and yeast cells of the pathogenic B. dermatitidis and in some oleaginous yeasts, e.g., the basidiogenous genera Rhodotorula and Rhodosporidium but less frequently in ascosporogenous yeasts. In S. cerevisiae ATCC 9896, long-chain fatty acids (C20-26), including 2-hydroxy-C26:0,42 accounted for about 15% of the plasmalemma fatty acids which were also present in the other membrane systems isolated. In both yeasts and filamentous fungi, such long-chain fatty acid components appear to be largely associated with sphingolipids.
Metabolic disorders, including glucose homeostasis and inborn errors of metabolism
Published in Janet M Rennie, Giles S Kendall, A Manual of Neonatal Intensive Care, 2013
Janet M Rennie, Giles S Kendall
Peroxisomes are subcellular organelles, and more than 15 different disorders of their function have been described. Several of these diseases present in the neonatal period, usually with severe neurological abnormality often combined with abnormal facies, hepatic failure and cataracts. Diagnosis is supported by high levels of very long chain fatty acids and/or deficiency of dihydroacetone phosphate acyl transferase in the plasma.
A review on neuropharmacological role of erucic acid: an omega-9 fatty acid from edible oils
Published in Nutritional Neuroscience, 2022
J. B. Senthil Kumar, Bhawna Sharma
Generally, lipid can be divided into five categories; fatty acids, triacylglycerols (TAGs), phospholipids, sterol lipids and sphingolipids. Fatty acids can be varied on the basis of length of carbon chain and degree of saturation. Fatty acid with no double bond in its structure is saturated fatty acids (SFAs) (e.g. butyric acid, myristic acid, palmitic acid, stearic acid, lauric acid, etc). Fatty acid having one double bond is known as monounsaturated fatty acid (MUFA) e.g. Oleic acid, erucic acid, palmitoleic acid, nervonic acid, etc and fatty acid with more than one double bond are said to be polyunsaturated fatty acid (PUFA) e.g. omega-3 fatty acid alpha linoleic acid, docosahexaenoic acid; omega-6 fatty acid-linoleic acid, gamma linoleic acid. On the basis of the carbon chain length, fatty acids can also be classified into short with less than 6 carbon atoms (e.g. acetic acid, butyric acid, etc), medium with 6–12 carbon atoms (e.g. caproic acid, lauric acid, etc), long chain fatty acid (LCFAs) with 13–20 carbon atoms and very long chain fatty acids (VLCFAs) with >20 or more carbon atoms.
Nutrient and Antioxidant Properties of Oils from Bagasses, Agricultural Residues, Medicinal Plants, and Fodders
Published in Journal of the American College of Nutrition, 2019
Agomuo Emmanuel Nnabugwu, Amadi Peter Uchenna
The long- and very-long-chain fatty acid contents of oils from some bagasse, agricultural residues, and forages are presented in Table 3. The arachidic and eicosanoic acid content of the oils evaluated were in a range of 0% to 12.27% and 0% to 13.11%, respectively. Only SJ and PP oils and PP and PM oils contained eicosadienoic and eicosatrienoic acids, respectively. CC oil contained the highest quantity of behenic acid followed by GH oil, while in the case of erucic and docosahexaenoic acid contents, GH oil showed higher compositions than CC oil. Further, the results in Table 3 showed that the range of lignoceric, nervonic, and cerotic acid compositions of the oils were in a range of 0% to 27.30%, 0% to 0.08%, and 0% to 10.29%, with the bagasse oils containing the highest quantities of lignoceric and cerotic acids but undetected nervonic acid contents. The reports of Ajayi (40) implied that African nutmeg contained comparable arachidic acids to those of the bagasse oils presented in this study, but the eicosanoic acid levels of most edible oils—coconut, sesame, and olive oils (39)—were lower than the oils analyzed in this study. Eicosanoic and eicosadienoic acids have reportedly been found to possess anti-inflammatory properties (41); hence, these oils could be evaluated for possible anti-inflammatory properties. Further, with high proportions of behenic acids, erucic acids, and other very-long-chain unsaturated fatty acids known for their cholesterol-elevating properties (42), CA, CC, and GH oils could only be suitable for non-food industrial uses, such as production of floor polishes and detergents.
Ophthalmic manifestations of Heimler syndrome due to PEX6 mutations
Published in Ophthalmic Genetics, 2018
Nutsuchar Wangtiraumnuay, Waleed Abed Alnabi, Mai Tsukikawa, Avrey Thau, Jenina Capasso, Reuven Sharony, Chris F. Inglehearn, Alex V. Levin
The peroxisome is a cytoplasmic organelle. Its main function is the breakdown of very long chain fatty acids through beta-oxidation. The PEX1 and PEX6 proteins bind with adenosine triphosphate (ATP) to form a heterohexameric ATPase which is associated with various cellular activities that fuel essential protein transport across peroxisomal membranes, the final steps of peroxisomal matrix-protein import (19–23). PEX1 and PEX6 are expressed in the retina, especially in photoreceptors (24). Abnormal PEX6 and PEX1 proteins result in abnormal peroxisomal function, leading to the accumulation of very long chain fatty acids. Histopathology of other peroxisomal disorders shows accumulation of characteristic bileaflet fatty acid inclusions in photoreceptors, RPE and pigment laden macrophages (13). Fatty acid accumulation may create the lipofuscin-like substances which appear as hyperfluorescent flecks seen on FAF in our patients.