BACKGROUND Liver transplantation for inherited metabolic disorders aims to save the patient's life when the disorder is expected to progress to organ failure, and to cure the underlying metabolic defect. METHODS We retrospectively analyzed 146 pediatric liver transplants (28 metabolic; 118 non-metabolic) performed between 1986 and 2000. RESULTS Twenty-eight transplants were performed in 24 ...

The analysis of urinary acylglycines is an important biochemical tool for the diagnosis of many organic acidemias and mitochondrial fatty acid β-oxidation defects. A new rapid analytical method has been developed for quantification of acylglycines in urine by liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS). The method requires a simple sample preparation avoiding derivati...

The effects of increased concentration of inorganic phosphate on the biosynthesis of tylosin, the level of the intracellular adenylates, the energy charge, and the activities of enzymes involved in the synthesis of tylonolide precursors were studied in Streptomyces fradiae NRRL 2702. No metabolic response was observed when elevated levels of inorganic phosphate were added in idiophase. Increase...

OBJECTIVES This study was performed to identify a target population of claudicants for propionyl-L-carnitine treatment. BACKGROUND Previous studies suggest that the efficacy of propionyl-L-carnitine in intermittent claudication is greater in patients with severe functional impairment than in those with mild walking disability. METHODS After run-in, 485 claudicant patients were randomized to...

1. A newly developed biotin assay was used to monitor plasma and urinary biotin concentration after the oral administration of biotin. 2. In two normal subjects, leucocyte propionyl-CoA carboxylase and beta-methylcrotonyl-CoA carboxylase were markedly stimulated after the administration of biotin.

BACKGROUND Marginal biotin deficiency may be a human teratogen. A biotin status indicator that is not dependent on renal function may be useful in studies of biotin status during pregnancy. A previous study of experimental biotin deficiency suggested that propionyl-coenzyme A carboxylase (PCC) activity in peripheral blood lymphocytes (PBLs) is a sensitive indicator of biotin status. OBJECTIVE...

In cyanobacteria, the CO2-concentrating mechanism (CCM) is a vital biological process that provides effective photosynthetic CO2 fixation by elevating the CO2 level near the active site of Rubisco. This process enables the adaptation of cyanobacteria to various habitats, particularly in CO2-limited environments. Although CCM of freshwater and marine cyanobacteria are well studied, there is limi...