混合二元酸中戊二酸的分离提纯

采用溶剂溶解和气泡结晶的方法从混合二元酸中分离、提纯戊二酸。考察了溶解温度、溶剂用量和结晶过程对戊二酸纯度和收率的影响 ,并确定了分离与提纯的最佳工艺条件。分离过程 :溶解温度 5 5℃、溶剂与二元酸用量比 30mL∶10g ;提纯过程 :结晶气速 5× 10 -6～ 8× 10 -6m3 /s、降温速率 1/ 3℃ /min、降温终点 2 0℃、结晶保温时间 2 0min、升温速率 1/ 2℃ /min、发汗终温 6 5℃、发汗保留时间 10min。在最佳工艺条件下分离、提纯得到的戊二酸纯度为 99 36 % ,戊二酸的回收率为 70 16 %。     

绿脓菌素促进铜绿假单胞菌NY3降解烃的作用机制

绿脓菌素（Pyo）的分泌是铜绿假单胞菌NY3最为显著的特征之一,前期实验观察到该菌降解烃时,Pyo分泌量常与烃的降解效率成正相关性,但其在污染物降解中的作用尚未受到关注,且机制不清楚.以共存戊二酸为高分泌Pyo的体系和试剂级Pyo标准物为基础,研究了NY3菌降解烃时,Pyo对细胞内氧化还原酶及其比酶活力和胞外电子传递速率等因素的影响作用.结果表明,戊二酸能够明显提高Pyo的分泌量,相较于无戊二酸体系提高了86.6%,且随Pyo分泌量增加,NY3菌对十六烷去除率增加了16.29%;NY3菌分泌Pyo有利于提高其胞内烷烃氧化酶的活力,在菌体生长至72h、Pyo投加量分别为200,300μL时,相较于未投加Pyo体系,烷烃氧化酶比活力分别提高了121.8%和346.5%.同时Pyo存在下将胞外电子传递速率提高了近7倍,从而增加其对十六烷的降解速率.NY3菌分泌Pyo能通过提高胞内酶活性和胞外电子传递速率而促进NY3菌降解十六烷.     

Novel mutation and prenatal sonographic findings of glutaric aciduria (type I) in two Taiwanese families

Glutaric aciduria type I (GA I) is an autosomal recessively inherited inborn error with a defect of the enzyme glutaryl-CoA dehydrogenase (GCDH), which has never been diagnosed prenatally in Taiwanese patients. We present the prenatal sonographic findings and mutational analysis data of three children in two Taiwanese families. One patient from each family was diagnosed postnatally due to macrocephaly and neurological deterioration at 4 months and 10 months, respectively. The third child, sister of the first patient, was diagnosed prenatally at 11 weeks' gestation through chorionic villus sampling (CVS). Molecular analysis revealed that the fetus and child in Family 1 were homozygous for a common mutation, IVS10 -2A>C, which has not been reported in the Caucasian population. The patient in Family 2 was a compound heterozygote for IVS10 -2A>C and a novel mutation 749T>C (L238P). After genetic counseling, the couple decided to continue the second pregnancy. However, dilatation of quadrigeminal cistern (QC) and suspicious macrocephaly were noted at 30 weeks. Progressive dilatation of the QC associated with macrocephaly, fronto-temporal atrophy and wide space of perisylvian fissure were found in the follow-up scans. The affected girl was delivered at 37 weeks' gestation by cesarean section. Postnatal magnetic resonance imaging (MRI) studies confirmed the prenatal sonographic findings. With prenatal sonographic findings and mutational analysis presented in the present cases, the feasibility of prenatal diagnosis of GA I in high-risk pregnancy can not be overlooked. Copyright © 2002 John Wiley & Sons, Ltd.     

Prenatal diagnosis of propionic acidemia

In this report we summarize our experience in prenatal diagnosis of propionic acidemia (PA) since 1987. Overall, we have investigated 25 pregnancies at risk from 19 unrelated families. Until genetic structure of the genes involved in PA was elucidated, prenatal diagnosis has been successfully performed by means of metabolite quantitation and/or enzymatic assays in foetal issue. Today, direct propionyl-CoA carboxylase activity assay in combination with molecular analysis in chorion villi can be regarded as a fast and reliable method of choice for prenatal diagnosis of this organic acidemia. Copyright © 2004 John Wiley & Sons, Ltd.     

戊二醛对紫色非硫光合细菌脱氢酶活力的影响

探讨微生物细胞对戊二醛毒性的耐受能力及应用条件的适宜范围,可为废水处理中固定化细胞的应用提供实验基础和理论依据。以紫色非硫光合细菌（PSB）为例,直接用戊二醛作用于细胞,在不同pH值、温度、戊二醛浓度、反应时间等条件下研究戊二醛对PSB脱氢酶活力的影响。结果表明：戊二醛对PSB脱氢酶活力影响的主要限制因素为戊二醛浓度和反应时间。当戊二醛浓度为0.5～1.0％,反应时间为1～2h时,PSB脱氢酶活力回收可达83％以上;PSB细胞经戊二醛处理后,热稳定性增强,pH稳定性无明显变化;并比较出热带假丝酵母菌和PSB对戊二醛的毒性耐受能力大于大肠埃希氏杆菌和芽孢杆菌yol。     

Prenatal diagnosis of propionic acidemia in chorionic villi by direct assay of propionyl CoA carboxylase

Prenatal diagnosis of propionic acidemia was achieved by the direct assay of propionyl CoA carboxylase in chorionic villi. The diagnosis was confirmed by determination of methylcitrate in amniotic fluid and measurement of propionyl CoA carboxylase in the liver from the abortion. Discrepancy between [¹⁴C]-propionate incorporation into protein of chorionic villi or cultured chorionic cells and propionyl CoA carboxylase activity is reported.