By using the laser-monitoring technique,solid-liquid equilibrium data of glutaric acid in cyclohexanol,cyclohexanone,their five mixed solvents and acetic acid were measured within the temperature range from 292.15K to...By using the laser-monitoring technique,solid-liquid equilibrium data of glutaric acid in cyclohexanol,cyclohexanone,their five mixed solvents and acetic acid were measured within the temperature range from 292.15K to 354.60K by dynamic method.Empirical formula and λh equation were used to correlate the solubilities of glutaric acid in eight solvents.The maximal average relative deviations were 1.15% and 2.20% by using the em-pirical formula and λh equation to correlate the solubility data.The results showed that the empirical formula and λh model could correlate the solubility data of glutaric acid in eight solvents.In addition,the solubility data of glutaric acid in five mixtures(cyclohexanone+cyclohexanol)could be predicted with the NRTL equation utilizing the pa-rameters of the binary systems.The total average relative deviation was 3.60%.The results indicate that the NRTL equation could well predict the solubilities of glutaric acid in the mixed solvents of cyclohexanone and cyclohexanol.展开更多
Glutaric acidemia type Ⅱ （GAⅡ）, also known as multiple acyl-CoA dehydrogenase defciency, is an auto-somal recessive inborn error of amino acid and fatty acid metabolism. We report a case of GAⅡ with novel electro...Glutaric acidemia type Ⅱ （GAⅡ）, also known as multiple acyl-CoA dehydrogenase defciency, is an auto-somal recessive inborn error of amino acid and fatty acid metabolism. We report a case of GAⅡ with novel electron transfer flavoprotein （ETF）-A mutations in a 2-year-old female with thalassemia minor. The patient developed an episode of hypoglycemia and hypotonicityon the postnatal first day. Laboratory investigations revealed elevations of multiple acyl carnitines indicat-ing glutaric acidemia type Ⅱ in newborn screening analysis. Urinary organic acids were evaluated for the confrmation and revealed a high glutaric acid excretion. Genetic analysis revealed two novel mutations in the ETF-A gene, which are considered to be compound heterozygote. At the 8 mo of life ketone therapy was added, which significantly increased the neuromotor development. The patient had been closely followed for two years with carnitine, ribofavin, coenzyme Q10, and ketone supplementation in addition to a high carbohydrate diet. Although the patient had comorbidity like thalassemia minor, her neuromotor developmentwas normal for her age and had no major health problems. This specific case expands the previously reported spectrum of this disease.展开更多
Fourteen novel compounds were synthesized and characterized by using NMR and ESI-MS methods. The bioactivities of the four novel 4-Ar-2-oxo-glutaric acids were studied by using the LC-MS method. The experimental resul...Fourteen novel compounds were synthesized and characterized by using NMR and ESI-MS methods. The bioactivities of the four novel 4-Ar-2-oxo-glutaric acids were studied by using the LC-MS method. The experimental results show that 3-nitrobenzyl-2-oxo-glutaric acid is a mild inhibitor for the hydroxylation reaction catalyzed by PHD2. The decarboxylated peak for 3-fluorobenzyl-2-oxo-glutaric acid was observed by using the negative LC-MS method, indicating that it can be used as a mild cosubstrate to replace 2-OG, but this is possible only in the presence of the prime peptide CODD 19 mer.展开更多
文摘By using the laser-monitoring technique,solid-liquid equilibrium data of glutaric acid in cyclohexanol,cyclohexanone,their five mixed solvents and acetic acid were measured within the temperature range from 292.15K to 354.60K by dynamic method.Empirical formula and λh equation were used to correlate the solubilities of glutaric acid in eight solvents.The maximal average relative deviations were 1.15% and 2.20% by using the em-pirical formula and λh equation to correlate the solubility data.The results showed that the empirical formula and λh model could correlate the solubility data of glutaric acid in eight solvents.In addition,the solubility data of glutaric acid in five mixtures(cyclohexanone+cyclohexanol)could be predicted with the NRTL equation utilizing the pa-rameters of the binary systems.The total average relative deviation was 3.60%.The results indicate that the NRTL equation could well predict the solubilities of glutaric acid in the mixed solvents of cyclohexanone and cyclohexanol.
文摘Glutaric acidemia type Ⅱ （GAⅡ）, also known as multiple acyl-CoA dehydrogenase defciency, is an auto-somal recessive inborn error of amino acid and fatty acid metabolism. We report a case of GAⅡ with novel electron transfer flavoprotein （ETF）-A mutations in a 2-year-old female with thalassemia minor. The patient developed an episode of hypoglycemia and hypotonicityon the postnatal first day. Laboratory investigations revealed elevations of multiple acyl carnitines indicat-ing glutaric acidemia type Ⅱ in newborn screening analysis. Urinary organic acids were evaluated for the confrmation and revealed a high glutaric acid excretion. Genetic analysis revealed two novel mutations in the ETF-A gene, which are considered to be compound heterozygote. At the 8 mo of life ketone therapy was added, which significantly increased the neuromotor development. The patient had been closely followed for two years with carnitine, ribofavin, coenzyme Q10, and ketone supplementation in addition to a high carbohydrate diet. Although the patient had comorbidity like thalassemia minor, her neuromotor developmentwas normal for her age and had no major health problems. This specific case expands the previously reported spectrum of this disease.
基金Supported by the Distinguished Scholars Fund of Jiangsu University(No06JDG050) the Natural Science Foundation ofAnhui Province(No2006KJ133B)
文摘Fourteen novel compounds were synthesized and characterized by using NMR and ESI-MS methods. The bioactivities of the four novel 4-Ar-2-oxo-glutaric acids were studied by using the LC-MS method. The experimental results show that 3-nitrobenzyl-2-oxo-glutaric acid is a mild inhibitor for the hydroxylation reaction catalyzed by PHD2. The decarboxylated peak for 3-fluorobenzyl-2-oxo-glutaric acid was observed by using the negative LC-MS method, indicating that it can be used as a mild cosubstrate to replace 2-OG, but this is possible only in the presence of the prime peptide CODD 19 mer.
