Cardiomyopathy and leukodystrophy are life-threatening complications of multiple acyl-CoA dehydrogenase deficiency (MADD). A 2-year-old boy with this disorder developed rapidly progressive leukodystrophy resulting in complete paralysis within 4 months. Within a week of starting sodium-D,L-3-hydroxybutyrate he had improved. After 2 years, neurological function returned, including walking indepen...

This assay for 3-hydroxybutyrate in plasma or serum, based on kinetic initial-rate techniques, has been optimized with respect to initial rate and linearity as follows: pH 8.5, hydroxybutyrate dehydrogenase 62.5 U/L, and NAD+ concentration 20 mmol/L. We have used the assay satisfactorily with both the Gilford 103 and Hitachi 705 discrete analyzers, obtaining results that compare well with those...

We describe two simple, rapid, and sensitive gas-chromatographic methods. One is for determining 3-hydroxybutyrate and lactate in plasma, the other for determining 3-hydroxybutyrate in perchloric acid extracts of blood. Samples are purified by adsorption onto graphitized carbon black (Carbopak B). After desorption, the two hydroxyacids are gas-chromatographically measured. Chromatography is on ...

Ketones can reactivate the production of fetal hemoglobin (HbF) in vitro and in vivo. A reactivation of HbF by ketones, which are generated during starvation, remains largely speculative. Therefore, we investigated HbF in 31 women with anorexia nervosa or bulimia, using both of these as models of intermittent starvation ketosis. For comparison, we also studied 42 female control subjects matched...

The archaeon Haloferax mediterranei was selected for production of PHA co- and terpolyesters using inexpensive crude glycerol phase (CGP) from biodiesel production as carbon source. CGP was assessed by comparison with the application of pure glycerol. Applying pure glycerol, a copolyester with a molar fraction of 3-hydroxybutyrate (3HB) of 0.90 mol/mol and 3-hydroxyvalerate (3HV) of 0.10 mol/mo...

Single crystals of biodegradable aliphatic polyesters, poly([R]-3-hydroxybutyrate), poly([R]-3-hydroxyvalerate), poly(L-lactic acid), poly( -propiolactone), poly(4-hydroxybutyrate), poly(Æ-valerolactone), poly("caprolactone), poly(ethylene succinate) and poly(tetramethylene adipate), were grown from dilute solution by isothermal crystallization, and the crystal structures and morphologies were ...

This is an automated method for the kinetic measurement of the combined concentrations of acetoacetate and beta-hydroxybutyrate in a single channel of the "Multistat III" centrifugal analyzer. Acetoacetate is first reduced with high concentrations of NADH by catalysis with 3-hydroxybutyrate dehydrogenase (EC 1.1.1.30). This reaction mixture is diluted with excess NAD+. The endogenous beta-hydro...

Halomonas sp. strain HG01, isolated from a salt mine in Peru, is a halophilic aerobic heterotrophic bacterium accumulating poly-3-hydroxybutyrate and poly(3-hydroxybutyrate-co-3-hydroxyvalerate) from different carbon sources. Here, we report the draft genome sequence of this isolate, which was found to be 3,665,487 bp long, with a G+C content of 68%.

BACKGROUND Ketone bodies are known to substitute for glucose as brain fuel when glucose availability is low. Ketogenic diets have been described as neuroprotective. Similar data have been reported for triheptanoin, a fatty oil and anaplerotic compound. In this study, we monitored the changes of energy metabolites in liver, blood, and brain after transient brain ischemia to test for ketone body ...