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Pathology of the Liver: Functional and Structural Alterations of Hepatocyte Organelles Induced by Cell Injury
Published in Robert G. Meeks, Steadman D. Harrison, Richard J. Bull, Hepatotoxicology, 2020
Louis Marzella, Benjamin F. Trump
Free radicals generated by hepatic metabolism or by drug biotransformation reactions can also cause cell damage by oxidation of unsaturated fatty acids of phospholipids. Lipid peroxidation is a feature of hepatocytes injured by toxins and by the accumulation of iron (Comporti, 1985; Hulcrantz et al., 1984). These reactions cause the autocatalytic breakdown of unsaturated fatty acids and the generation of fatty aldehydes and other carboxyl compounds which can in turn react with SH groups and covalently bind to proteins (Comporti, 1985). Altered membrane permeability or inactivation of critical membrane pumps can result from lipid peroxidation (Lowrey et al., 1981). The quantitative importance of lipid peroxidation in the evolution of cell death is undetermined. The prevailing opinion is that peroxidation is not an early event in cell injury such as that induced by ischemia (Silver and Szabo, 1983).
Metabolomics in amniotic fluid
Published in Moshe Hod, Vincenzo Berghella, Mary E. D'Alton, Gian Carlo Di Renzo, Eduard Gratacós, Vassilios Fanos, New Technologies and Perinatal Medicine, 2019
Alexandra-Maria Michaelidou, Foteini Tsakoumaki, Maria Fotiou, Charikleia Kyrkou, Apostolos P. Athanasiadis
According to Gil and Duarte (12), two studies (53,54) have addressed AF collected at the time of birth, in order to delineate a metabolic picture of the PTB newborn at that time. The changes recorded were in support of the large impact of PTB on AF metabolic profile and were mainly associated with amino acids, unsaturated fatty acids, oxylipins, fatty aldehydes, and choline compounds.
Ichthyotic disorders
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Sjögren-Larsson syndrome (SLS) is an autosomal recessive disorder characterized by clinical symptoms of congenital ichthyosis, spastic paresis, and mental retardation. SLS is caused by mutation in the gene fatty aldehyde dehydrogenase. SLS presents at birth or in the neonatal period with varying degrees of erythema and ichthyosis. The collodion phenotype is rarely seen. Erythema is seen at birth but may not be evident after 1 year of age. Ichthyosis is usually fine to lamellar type and predominantly affects the lower abdomen, sides, and nape of neck and flexures (Figure 22.9) [12]. PPK is seen in 50% of cases. Neurological features include speech and motor delay, spastic paresis, and seizures. One-third of patients present with foveal and perifoveal glistening dots that appear after several years of age. Bilateral glistening yellow white dots involving the foveal and parafoveal regions are present from the age of 1–2 years. The number of dots increases with age, although the extent of maculae involvement does not correlate with systemic severity. Diagnosis is confirmed by the measurement of enzyme activity in cultured skin fibroblasts or leucocytes.
Protective action of alkylglycerols under stress
Published in Stress, 2020
Tatiana S. Poleschuk, Ruslan M. Sultanov, Ekaterina V. Ermolenko, Lidiya V. Shulgina, Sergey P. Kasyanov
Under oxidative stress, changes in the mitochondrial membrane potential and permeabilization (or rupture) of the outer membrane lead to release of cytochrome c. Cytochrome c possesses a high redox potential (+260 mV) and a compact tertiary structure containing covalently bound heme iron. Upon interaction with negatively charged phospholipids, especially cardiolipin, cytochrome c undergoes a conformational alteration with subsequent displacement of the axial Met-80 that is accompanied by a dramatic decrease in its redox potential to –400 mV. Cardiolipin-activated cytochrome c (or plasmalogenase) catalyzes the oxidatively enabled hydrolytic cleavage of the vinyl ether linkage of plasmenylcholine and plasmenylethanolamine in the presence of hydrogen peroxide (H2O2). The reaction products are 2-acyl-lysophospholipids and highly reactive α-hydroxy fatty aldehydes (Jenkins et al., 2018).
Sjögren-Larsson syndrome: a complex metabolic disease with a distinctive ocular phenotype
Published in Ophthalmic Genetics, 2019
Samiksha Fouzdar-Jain, Donny W Suh, William B Rizzo
FALDH functions as a solitary protein acting on fatty aldehydes and as a critical component of the fatty alcohol: nicotinamide adenine dinucleotide oxidoreductase (FAO) enzyme complex that, together with alcohol dehydrogenase, sequentially oxidizes fatty alcohols to fatty acids (4,32) (Figure 2). Defects in FALDH and FAO, therefore, result in impaired oxidation of both fatty aldehydes and alcohols (33). FALDH is also involved in metabolism of fatty aldehydes that are produced from several diverse lipid pathways (34) (Figure 2). These pathways include degradation of 2-hexadecenal produced by cleavage of sphingosine-1-phosphate (35), oxidation of pristanal derived from the dietary branched-chain fatty acid phytanic acid (36,37), metabolism of ω-hydroxy-fatty acids including leukotriene B4 (38), fatty aldehydes produced from catabolism of ether glycerolipids (39), and certain aldehyde byproducts of oxidative stress (40,41). Consequently, FALDH has a central role in eliminating a variety of fatty aldehydes that arise during lipid metabolism.
Change of plasmalogen content of red blood cells in myocardial hypoxia and acidosis
Published in Acta Cardiologica, 2018
Alexander Nikolaevich Osipenko
Subsequently, we have conducted an analysis of various aldehydogenic alkenyl (fatty aldehydes) and acyl (fatty acids) groups of lipid molecules which were present in hexane extracts in the forms of diethyl acetals and ethyl esters. It should be noted that in many contemporary articles, the term fatty alcohol (similarly with vinyl alcohol) is used to denote an alkenyl group of plasmalogens [3–5,7]. However, aldehydes (with subsequent formation of acetals) are released from plasmalogens during acid hydrolysis, which is usually used when analysing fatty acids. In this connection, in other articles, the term fatty aldehydes is used [13–16].