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Carnitine-acylcarnitine translocase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Dynamic acetylation and deacetylation of nuclear histones is essential for regulating the access of chromosomal DNA to transcriptional machinery. The source of acetylCoA for histone acetylation in mammalian cell nuclei is not clear. Acetylcarnitine formed in mitochondria is transported into cytosol by carnitine/acylcarnitine translocase, and then enters the nucleus, where it is converted to acetylCoA by a nuclear carnitine acetyltransferase and becomes a source of acetyl groups for histone acetylation. Genetic deficiency of the translocase markedly reduced the mitochondrial acetylcarnitine-dependent nuclear histone acetylation, indicating the significance of the carnitine-dependent mitochondrial acetyl group contribution to histone acetylation [39].
Micronutrients in Healthy Aging and Age-Related Decline in Organ Functions
Published in Kedar N. Prasad, Micronutrients in Health and Disease, 2019
A combination of alpha lipoic acid and acetyl-L-carnitine reduced mitochondrial decay and oxidative damage in rat brain during aging.165 These results support many other studies discussed previously that oxidative damage of mitochondria plays a central role in increasing the risk of age-related chronic diseases and organ functional deficits. Alpha-lipoic acid and acetyl-L-carnitine are substrates for mitochondrial enzyme carnitine acetyltransferase (CAT). In the brains of older rats, the activity of CAT and its binding affinity with substrates declined compared to younger rats.166 Feeding old rats with high doses of acetyl-L-carnitine and alpha-lipoic acid can ameliorate oxidative damage, carnitine acetyltransferase activity and its binding affinity with substrates, and mitochondrial dysfunction.166
Fungal Lipids
Published in Rajendra Prasad, Mahmoud A. Ghannoum, Lipids of Pathogenic Fungi, 2017
The amount of lipid present in fungal cultures ranges from < 5% to over 25% of biomass, varying with stage of development, culture conditions and species. A study of 12 species of Mortierella found that all of them were active producers of lipid which could account for 40-60% of biomass.16 Oleaginous fungi, defined as capable of accumulating more than 20% of their dry weight as lipid, include species of Lipomyces in which the lipid content may reach 40%. Conversion of such lipid reserves into new biomass has been demonstrated in eight strains of the oleaginous yeasts, Candida curvata, Lipomyces starkeyi, Rhodosporidium toruloides and Trichosporum cutaneum, transferred to carbon starvation conditions after accumulation of up to 34% of biomass as lipid.17 During this lipid utilization, the activities of ATP: citrate lyase (EC 4.1.3.8) and malate dehydrogenase (EC 1.1.1.42) decreased, while those of carnitine acetyltransferase (EC 2.3.1.7) and isocitrate lyase (EC 4.1.3.1) increased.18 The oleaginicity of yeasts and filamentous fungi can be correlated with ATP-citrate lyase activity19 and has been reviewed by Ratledge.20
Logistic role of carnitine shuttle system on radiation-induced L-carnitine and acylcarnitines alteration
Published in International Journal of Radiation Biology, 2022
L-carnitine (β-hydroxy-γ-trimethylammonium butyrate) as a carrier of the carnitine shuttle system which is comprised of the carnitine palmitoyltransferase 1 (CPT1) and 2 (CPT2), the carnitine-acylcarnitine translocase (CACT), and the carnitine acetyltransferase (CrAT) to transport long-chain fatty acids from the cytosol into mitochondria (Ramsay et al. 2001; Virmani et al. 2015; Houten et al. 2016). The carnitine shuttle system controls the flux of fatty acid β-oxidation (FAO). Long-chain (C14–C20) fatty acid acyl groups are transported exclusively as carnitine esters by the carnitine shuttle system. In contrast, the short- (C2–C5) and medium-chain fatty acids are transported into the mitochondrial matrix without any carnitine assistance in the process. In addition, most of the acylcarnitines, which are esters of L-carnitine and acyl groups, are derived from the intermediates of FAO. The alteration of the carnitine pool comprised free L-carnitine and various acylcarnitines in blood and urine might represent the changing flux of FAO.
Carnitine is a pharmacological allosteric chaperone of the human lysosomal α-glucosidase
Published in Journal of Enzyme Inhibition and Medicinal Chemistry, 2021
Roberta Iacono, Nadia Minopoli, Maria Carmina Ferrara, Antonietta Tarallo, Carla Damiano, Caterina Porto, Sandra Strollo, Véronique Roig-Zamboni, Gianfranco Peluso, Gerlind Sulzenbacher, Beatrice Cobucci-Ponzano, Giancarlo Parenti, Marco Moracci
The use of L-carnitine as a drug for the treatment of PD is particularly attractive. L-CAR is involved in fatty acid metabolism and synthesised mainly in the liver and kidneys from the essential amino acids lysine and methionine as ultimate precursors to form trimethyl lysine. L-CAR is not toxic at the concentration normally administrated and its use is approved as nutraceutical. Instead, the use of D-carnitine and acetyl-D-carnitine in clinics is less reliable. D-Carnitine can interfere with the uptake and transport of L-carnitine by inhibiting the carnitine acetyltransferase and its use in patients affected by kidney illnesses is avoided49. In addition, documented clinical use of acetyl-D-carnitine and its pharmacologically acceptable salts is limited to the therapeutic treatment of glaucoma63. Therefore, L-CAR might be promptly included in clinical protocols for the treatment of PD while its D-CAR and A-D-CAR derivatives need more investigations.
Comparison of the therapeutic effects of erythropoietin and acetyl-l-carnitine on sciatic nerve injury in rats
Published in Neurological Research, 2022
Ceren Kencebay Manas, Narin Derin, Ramazan Yavuz Arican, Gamze Tanriover, Sayra Dilmac, Haluk Ozcanli
Another bioactive molecule, acetyl-L-carnitine (ALCAR), the acetylated derivative of carnitine, is synthesized by carnitine acetyl-transferase, which catalyzes the acetylation of carnitine in mitochondria. This molecule transports long-chain fatty acids across the mitochondrial membrane, causing oxidation, thereby regulating cellular energy metabolism [6]. Several studies have reported the antioxidant and anti-inflammatory properties of ALCAR [7].