3-methylbutyrylcarnitine and propionylcarnitine

Carnitine and its derivatives are natural substances involved in both carbohydrate and lipid metabolism. This review summarizes the recent progress in the field in relation to the molecular mechanisms.. The pool of different carnitine derivatives is formed by acetyl-L-carnitine (ALC), propionyl-L-carnitine (PLC), and isovaleryl-carnitine. ALC may have a preferential effect on the brain tissue. ALC represents a compound of great interest for its wide clinical application in various neurological disorders: it may be of benefit in treating Alzheimer's dementia, depression in the elderly, HIV infection, chronic fatigue syndrome, peripheral neuropathies, ischemia and reperfusion of the brain, and cognitive impairment associated with various conditions. PLC has been demonstrated to replenish the intermediates of the tricarboxylic acid cycle by the propionyl-CoA moiety, a greater affinity for the sarcolemmal carrier, peripheral vasodilator activity, a greater positive inotropism, and more rapid entry into myocytes. Most studies of the therapeutic use of PLC are focused on the prevention and treatment of ischemic heart disease, congestive heart failure, hypertrophic heart disease, and peripheral arterial disease. ALC and PLC are considered well tolerated without significant side-effects.. A number of therapeutic effects possibly come from the interaction of carnitine and its derivatives with the elements of cellular membranes.

Topics: Acetylcarnitine; Cardiotonic Agents; Cardiovascular Diseases; Carnitine; Cognition Disorders; Depression; Diabetes Mellitus; Humans; Liver Cirrhosis; Nervous System Diseases; Nootropic Agents

To determine the plasma carnitine ester profile in adult patients with ulcerative culitis (UC) and compared with healthy control subjects.. Using ESI triple quadrupole tandem mass spectrometry, the carnitine ester profile was measured in 44 patients with UC and 44 age- and sex-matched healthy controls.. There was no significant difference in the fasting free carnitine level between the patients with UC and the healthy controls. The fasting propionyl- (0.331 +/- 0.019 vs 0.392 +/- 0.017 micromol/L), butyryl- (0.219 +/- 0.014 vs 0.265 +/- 0.012), and isovalerylcarnitine (0.111 +/- 0.008 vs 0.134 +/- 0.008) levels were decreased in the UC patients. By contrast, the level of octanoyl-(0.147 +/- 0.009 vs 0.114 +/- 0.008), decanoyl- (0.180 +/- 0.012 vs 0.137 +/- 0.008), myristoyl- (0.048 +/- 0.003 vs 0.039 +/- 0.003), palmitoyl- (0.128 +/- 0.006 vs 0.109 +/- 0.004), palmitoleyl- (0.042 +/- 0.003 vs 0.031 +/- 0.002) and oleylcarnitine (0.183 +/- 0.007 vs 0.163 +/- 0.007; P < 0.05 in all comparisons) were increased in the patients with UC.. Our data suggest selective involvement of the carnitine esters in UC patients, probably due to their altered metabolism.

Topics: Adolescent; Adult; Aged; Carnitine; Colitis, Ulcerative; Fasting; Fatty Acids; Female; Humans; Male; Middle Aged

Concentrations of l-carnitine and acylcarnitines have been determined in urine from patients with disorders of organic acid metabolism associated with an intramitochondrial accumulation of acyl-CoA intermediates. These included propionic acidemia, methylmalonic aciduria, isovaleric acidemia, multicarboxylase deficiency, 3-hydroxy-3-methylglutaric aciduria, methylacetoacetyl-CoA thiolase deficiency, and various dicarboxylic acidurias including glutaric aciduria, medium-chain acyl-CoA dehydrogenase deficiency, and multiple acyl-CoA dehydrogenase deficiency. In all cases, concentrations of acylcarnitines were greatly increased above normal with free carnitine concentrations ranging from undetectable to supranormal values. The ratios of acylcarnitine/carnitine were elevated above the normal value of 2.0 +/- 1.1. l-Carnitine was given to three of these patients; in each case, concentrations of plasma and urine carnitines increased accompanied by a marked increase in concentrations of short-chain acylcarnitines. These acylcarnitines have been examined using fast atom bombardment mass spectrometry in some of these diseases and have been shown to be propionylcarnitine in methylmalonic aciduria and propionic acidemia, isovalerylcarnitine in isovaleric acidemia, and hexanoylcarnitine and octanoylcarnitine in medium-chain acyl-CoA dehydrogenase deficiency. The excretion of these acylcarnitines is compatible with the known accumulation of the corresponding acyl-CoA esters in these diseases. In this group of disorders, the increased acylcarnitine/carnitine ratio in urine and plasma indicates an imbalance of mitochondrial mass action homeostasis and, hence, of acyl-CoA/CoA ratios. Despite naturally occurring attempts to increase endogeneous l-carnitine biosynthesis, there is insufficient carnitine available to restore the mass action ratio as demonstrated by the further increase in acylcarnitine excretion when patients were given oral l-carnitine.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acyl-CoA Dehydrogenases; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Carnitine; Child; Child, Preschool; Female; Hemiterpenes; Humans; Infant; Infant, Newborn; Lysine; Male; Methylmalonic Acid; Middle Aged; Pentanoic Acids; Propionates