Polyhydroxyalkanoates (PHA) is a family of microbially synthesized polyesters consisting of various 3-hydroxyalkanoate monomers. Aeromonas hydrophila 4AK4 could be able to synthesize PHA copolymer consisting of 3-hydroxybutyrate (3-HB) and 3-hydroxyhexanoate (3-HHx). No data has been reported about the ability to synthesize the PHA with other monomers in A. hydrophila. In this study, propionic acid, valeric acid, heptanoic acid, nonanoic acid and undecanoic acid were used together with gluconate to find out whether A. hydrophila 4AK4 could synthesize the PHA consisting of odd carbon atom number monomers. The result showed that A. hydrophila 4AK4 could not growth when supplied with propionic acid, valeric acid, heptanoic acid and nonanoic acid and only undecanoic acid could be used to synthesize PHA. Wild type and recombinant A. hydrophila 4AK4 harboring phaA (beta-ketothiolase) and phaB (acetoacetyl-CoA reductase) were cultivated with undecanoic acid and glucose or undecanoic acid and gluconate served as carbon sources. PHA consisting of 3-HB and 3-hydroxyvalerate (3-HV) could be produced by both wild type and recombinant A. hydrophila 4AK4 and the latter could produce PHA with more 3-HB monomer. When the ratio of glucose or gluconate to undecanoic acid was 1:1, the cell dry weight (CDW) of A. hydrophila 4AK4 reached 1.14 g/L and PHA content was 60% of the CDW after cultivation for 24 h. When lauric acid and undecanoic acid were served as co-substrate, A. hydrophila 4AK4 could produce copolyester consisting of 3-HB, 3-HV and 3-HHx. Along with the increase of undecanoic acid proportion in the mixed carbon source, the 3-HV content of copolymer was increased while the 3-HB and 3-HHx content were decreased. In all cases, the CDW decreased along with the increase of undecanoic acid concentration, which indicated that undecanoic acid was not very good for A. hydrophila 4AK4 growth.
Aeromonas hydrophila ; metabolism ; Fatty Acids ; metabolism ; Glucose ; metabolism ; Lauric Acids ; metabolism ; Pentanoic Acids ; metabolism ; Polyhydroxyalkanoates ; biosynthesis

Ethyl (R)-3-hydroxy-5-(1,3-dioxoisoindolin-2-yl)-pentanoate is a potential intermediate for the synthesis of HMG-CoA reductase inhibitor (atorvastatin) that can lower the cholesterol level in human blood. In this study, in order to synthesize ethyl (R)-3-hydroxy-5-(1,3-dioxoisoindolin-2-yl)-pentanoate by bioreduction, the yeast strains in our lab were screened. Ethyl (R)-3-hydroxy-5-(1,3-dioxoisoindolin-2-yl)-pentanoate was found to be produced efficiently from ethyl 5-(1,3-dioxoisoindolin-2-yl)-3-oxopentanoate by Pichia pastoris X-33. The effects of initial substrate concentration, reaction time, co-substrate, amount of yeast cells, pH, as well as the temperature on the yield and enantiomeric excesses (e.e. value) of product were examined in mono-phase system. The optimal reaction conditions are as fallows: substrate concentration 7 g/L, cell concentration 120 g/L, glucose concentration 120 g/L, pH 6.5, temperature 35 degrees C, reaction time 12 h, and the yield 93.12% with the high e.e. value of 98.55%.
Anticholesteremic Agents ; metabolism ; Atorvastatin Calcium ; Catalysis ; Enzymes ; metabolism ; Fermentation ; Heptanoic Acids ; metabolism ; Isoindoles ; metabolism ; Oxidation-Reduction ; Pentanoic Acids ; metabolism ; Pichia ; genetics ; metabolism ; Pyrroles ; metabolism ; Stereoisomerism

OBJECTIVEThe diagnosis of organic acidemia is very difficult and needs special test methods. Recently the tandem mass spectrometry has been used in screening for and diagnosis of this inborn error of metabolism. The aim of the present study was to utilize a dry blood filter paper method for acylcarnitines profiles test using tandem mass spectrometry in diagnosis of organic acidemias in high risk children.

METHODOne thousand patients (642 were males and 358 females) with high risk of inborn error of metabolism were studied, the median of their age was 2 years. The blood specimens were collected on filter paper, punched and extracted into methanol solution with stable isotope labeled internal standards of acylcarnitine, then derivatized with butanolic-HCI. After preparation, the samples were analyzed by tandem mass spectrometry. The volume of the acylcarnitines was calculated with special software.

RESULTSForty patients (4.0%) were diagnosed as organic acidemias among the 1000 patients, including 20 methylmalonic acidemia, 6 propionic acidemia, 3 isovaleric acidemia, 3 glutaric acidemia type I, 3 glutaric acidemia type II, 2 biotinidas deficiency, 1 3-methylcrotonyl-CoA carboxylase deficiency, 1 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, and 1 beta-keto thiolase deficiency. The common clinical symptoms and signs of these patients were motor and mental developmental retardation, spasm, lethargy, coma, hypotonia, vomiting, and feeding difficulty. Routine laboratory tests suggested metabolic acidosis, hyperammonemia, hyperlactacidemia, hypoglycemia, anemia, and abnormal liver function.

CONCLUSIONA part of organic acidemias can be rapidly diagnosed by tandem mass spectrometry through acylcarnitine profiles analysis in dry blood filter paper. Combination of tandem mass spectrometry with urine gas chromatography mass spectrometry would improve more accurate diagnosis of organic acidemias.

Adolescent ; Child ; Child, Preschool ; Female ; Filtration ; Gas Chromatography-Mass Spectrometry ; Glutarates ; blood ; Humans ; Infant ; Male ; Metabolism, Inborn Errors ; diagnosis ; Methylmalonic Acid ; blood ; Pentanoic Acids ; blood ; Propionic Acidemia ; diagnosis ; Tandem Mass Spectrometry ; methods

No abstract available.
Valproic Acid*

No abstract available.
Animals ; Mice* ; Valproic Acid*

Valproic acid intoxication is a fairly common clinical problem that can result in serious complications. Traditionally the treatment of valproic acid overdose has been limited to supportive measures, but high blood levels may require extracorporeal removal, and publications on this experience are scarce. This case demonstrated continuous veno-venous hemodiafiltration successfully used in patient with severe valproic acid overdose who was hemodynamically unstable.
Hemodiafiltration ; Humans ; Valproic Acid

No abstract available.
Erythema Nodosum* ; Erythema* ; Valproic Acid*

No abstract available.
Bipolar Disorder* ; Hair* ; Valproic Acid*

No abstract available.
Bipolar Disorder ; Hair ; Valproic Acid

PURPOSE: Epileptic patients under prolonged treatment with anticonvulsants such as valproate tend to have low serum carnitine level. The object of this study is to observe the interrelationship between anticonvulsants and serum carnitine level and its related symptoms. METHODS: We measured the serum carnitine levels in 31 epileptic patients (18 males, 13 females) receiving diverse antiepileptic drugs and 32 healthy children (20 males, 12 females) using enzymatic cycling method. RESULTS: 1) Among the control group, total, free, and acyl-carnitine in serum were 50.04+/-11.44, 37.42+/-11.13, and 13.98+/-7.49micro mol/L (mean+/-S.D.), respectively, and no significant differences were observed between age and sex. 2) Among 31 patients with treatment, total serum carnitine level and free carnitine level below 2 standard deviation from the mean control value was not observed. 3) Among 31 patients with treatment, total serum carnitine level below 2 standard deviation from the age matched mean control value was not observed. 4) No significant relationship was observed between the serum carnitine level and the dosage, duration and the serum concentration of the antiepileptic drugs. 5) There were no abnormalities in serum AST, ALT levels among the patients treated. CONCLUSION: Although our study didn't reveal that serum carnitine level can be lowered in patients treated with anticonvulsants such as valproate, many others reported it. So periodic measurement of serum carnitine level before and after treatment with anticonvulsants should be considered.
Anticonvulsants* ; Carnitine* ; Child* ; Humans ; Male ; Valproic Acid