Glutaric aciduria type I (glutaryl-CoA dehydrogenase deficiency) is an inborn error of metabolism that usually manifests in infancy by an acute encephalopathic crisis and often results in permanent motor handicap. Biochemical hallmarks of this disease are elevated levels of glutarate and 3-hydroxyglutarate in blood and urine. The neuropathology of this disease is still poorly understood, as low...

The mechanisms underlying ethylmalonic-adipic aciduria were studied in a 5-yr-old girl. Oxidation of radioactive substrates by cultured skin fibroblasts from the proband and asymptomatic family members was also determined and compared to that by normal fibroblasts and that by cells from a patient with glutaric aciduria type II. Feeding medium-chain triglycerides promptly induced vomiting and le...

The possibility of using the nutritionally versatile bacterium Pseudomonas cepacia to produce poly-beta-hydroxyalkanoic acid was evaluated. Chemostat culture showed that growth of P. cepacia became nitrogen limited when the molar carbon-to-nitrogen ratio of the medium fed into the fermentor was above 15. When grown under nitrogen limitation in batch culture with fructose as the sole source of c...

A semisynthetic aminoglycoside antibiotic 15, containing a cyclic r-amino-a-hydroxy acid, related to the 1-N-4-amino-2-hydroxybutyric acid (AHBA) side chain of butirosins and amikacin, has been prepared. Conveniently protected 3,2',6'-tris-N-tert-butoxycarbonylgentamicin C,a, (12) was condensed with the phtalimido active ester 10 to give after catalytic reduction and deprotection, the hitherto ...

-Hydroxybutyric acid (GHB) is a therapeutic drug, a drug of abuse, and an endogenous substance that binds to lowand high-affinity sites in the mammalian brain. To target the specific GHB binding sites, we have developed a I-labeled GHB analog and characterized its binding in rat brain homogenate and slices. Our data show that [I]4-hydroxy-4-[4-(2-iodobenzyloxy)phenyl]butanoate ([I]BnOPh-GHB) bi...

Staphylococcus sp. strain BP/SU1, capable of degrading the biopolymer and utilize it as a source of carbon and energy, was isolated from activated sludge using METABOLIX (MBX D411G). It was found that this strain was capable of accumulating poly(3-hydroxybutyric acid) P(3-HB), as granule poly (3-hydroxybutyric acid), p(3-HB), inclusion bodies when grown under suitable nutrient conditions. These...