Disease relevance of [(2R)-2-amino-3-carboxy-propyl]-trimethyl-ammonium

• L-carnitine partly abolished the PA-mediated growth reduction of glioma cells, whereas etomoxir and aminocarnitine enhanced the antiproliferative effect of PA [1].
• L-carnitine protection in ammonia intoxication. Effect of aminocarnitine on carnitine-dependent metabolism and acute ammonia toxicity [2].
• Effects of refeeding diets on emeriamine-induced fatty liver in fasting rats [3].
• These results suggest that specific inhibition of fatty acid oxidation by emeriamine diverted the exogenous fatty acid to the esterification pathway, and induced fatty liver and hypertriglyceridemia under fasting conditions [4].
• Emeriamine (10 mg/kg body weight) was orally administered to rats under the two different physiological conditions of a 2-day fast or refeeding with a high-carbohydrate diet after a 2-day fast [4].

High impact information on [(2R)-2-amino-3-carboxy-propyl]-trimethyl-ammonium

• Here we show that decanoyl-DL-aminocarnitine and palmitoyl-DL-aminocarnitine inhibit carnitine palmitoyltransferase in vitro about 7-fold and 100-fold more effectively than does aminocarnitine [5].
• DL-Aminocarnitine and, to a lesser extent, acetyl-DL-aminocarnitine are also inhibitors of the carnitine palmitoyltransferase activity of detergent-lysed rat liver mitochondria; in the presence of 1 mM L-carnitine, 5 microM aminocarnitine inhibits palmitoyl transfer by 64% [6].
• As anticipated from the low group transfer potential of amides, carnitine acetyltransferase catalyzes the transfer of acetyl groups from CoASAc to aminocarnitine (Km = 3.8 mM) but does not catalyze detectable transfer from acetylaminocarnitine to CoASH [6].
• Aminocarnitine (3) is a weak competitive inhibitor (K(i) = 4.0 mM) for carnitine acetyltransferase (CAT), although the N-acetyl derivative 4 is about 165 times more potent (K(i) = 0.024 mM) than 3 [7].
• Both mercaptoacetate (400 or 600 micromol/kg) and emeriamine (34.8 or 69.6 micromol/kg) significantly increased food intake [8].

Biological context of [(2R)-2-amino-3-carboxy-propyl]-trimethyl-ammonium

• (2) Inhibition of hepatic fatty acid oxidation: beta-aminobetaine (emeriamine) is a water-soluble carnitine analog and inhibition of CPT-1 in isolated hepatocytes is 100 times more sensitive than that in isolated cardiocytes and it suppresses both gluconeogenesis and ketogenesis by 60-80% [9].
• The effect of emeriamine, a potent inhibitor of the entry of fatty acids into mitochondria on lipid metabolism, was examined [4].

Associations of [(2R)-2-amino-3-carboxy-propyl]-trimethyl-ammonium with other chemical compounds

• DL-Aminocarnitine (3-amino-4-trimethylaminobutyric acid) and acetyl-DL-aminocarnitine (3-acetamido-4-trimethylaminobutyric acid) have been synthesized and the interactions of these compounds with carnitine acetyltransferase and carnitine palmitoyltransferase investigated [6].
• Liver perfusion with increasing concentrations of emeriamine up to 3 mumol caused a dose-dependent inhibition of ketone body production [10].

Gene context of [(2R)-2-amino-3-carboxy-propyl]-trimethyl-ammonium

• Rats treated orally for 21 days with aminocarnitine, an inhibitor of carnitine palmitoyltransferase-2 (CPT-2), do not show hypertrophy of the heart [11].

References