人体中R-和S-甲基丙二酸半醛相互转化的示踪研究。
Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man.
作者信息
Manning N J, Pollitt R J
出版信息
Biochem J. 1985 Oct 15;231(2):481-4. doi: 10.1042/bj2310481.
Abstract
Two human subjects were given separate oral doses of sodium [2H6]isobutyrate and [methyl-2H3]thymine and the labelling patterns of urinary metabolites were determined. Ingestion of deuterated isobutyrate resulted in the excretion of 2H5-labelled S-3-hydroxyisobutyric acid, formed on the direct catabolic pathway, and of S- and R-[2H4]-3-hydroxyisobutyric acids, formed by the reduction of S- and R-methylmalonic semialdehydes respectively. Only the R-enantiomer of urinary 3-hydroxyisobutyric acid was labelled by thymine. This labelling pattern indicates a flow from S- to R-methylmalonic semialdehyde, suggesting that the R-enantiomer is the substrate of methylmalonic semialdehyde dehydrogenase.
摘要
给两名人类受试者分别口服[2H6]异丁酸钠和[甲基-2H3]胸腺嘧啶，并测定尿液代谢物的标记模式。摄入氘代异丁酸盐导致直接分解代谢途径上形成的2H5标记的S-3-羟基异丁酸以及分别由S-和R-甲基丙二酸半醛还原形成的S-和R-[2H4]-3-羟基异丁酸的排泄。只有尿液中3-羟基异丁酸的R-对映体被胸腺嘧啶标记。这种标记模式表明从S-甲基丙二酸半醛流向R-甲基丙二酸半醛，表明R-对映体是甲基丙二酸半醛脱氢酶的底物。
相似文献
1
Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man.人体中R-和S-甲基丙二酸半醛相互转化的示踪研究。
Biochem J. 1985 Oct 15;231(2):481-4. doi: 10.1042/bj2310481.
2
Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.β-丙氨酸、3-羟基丙酸、R-和S-3-氨基异丁酸、R-和S-3-羟基异丁酸以及S-2-(羟甲基)丁酸排泄过多，可能是由于相应丙二酸半醛代谢缺陷所致。
J Inherit Metab Dis. 1985;8(2):75-9. doi: 10.1007/BF01801669.
3
4
Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry.采用对映体选择性多维毛细管气相色谱-质谱法对人尿中3-羟基异丁酸和3-氨基异丁酸进行立体化学鉴别。
Clin Chim Acta. 2000 Feb 25;292(1-2):93-105. doi: 10.1016/s0009-8981(99)00210-7.
5
Hypermethioninaemia and 3-hydroxyisobutyric aciduria in an apparently healthy baby.一名看似健康的婴儿出现高甲硫氨酸血症和3-羟基异丁酸尿症。
J Inherit Metab Dis. 1981;4(2):79-80. doi: 10.1007/BF02263600.
6
Isolation and identification of S(+)-3-hydroxyisobutyric acid in the urine of rats loaded with isobutyric acid.异丁酸负荷大鼠尿液中S(+)-3-羟基异丁酸的分离与鉴定
Biochim Biophys Acta. 1979 Jul 18;585(4):643-4. doi: 10.1016/0304-4165(79)90196-x.
7
Methylmalonic semialdehyde dehydrogenase deficiency: psychomotor delay and methylmalonic aciduria without metabolic decompensation.甲基丙二酸半醛脱氢酶缺乏症：精神运动发育迟缓与无代谢失代偿的甲基丙二酸尿症
Mol Genet Metab. 1998 Sep;65(1):35-43. doi: 10.1006/mgme.1998.2737.
8
Isobutyrate as a precursor of n-butyrate in the biosynthesis of tylosine and fatty acids.异丁酸盐作为泰乐菌素和脂肪酸生物合成中丁酸盐的前体。
FEMS Microbiol Lett. 1991 Nov 1;68(1):33-6. doi: 10.1016/0378-1097(91)90390-v.
9
Metabolism of malonic semialdehyde in man.人体内丙二酸半醛的代谢
Biochem J. 1983 Oct 15;216(1):81-5. doi: 10.1042/bj2160081.
10
Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.一种新的产生R系列代谢产物的哺乳动物异亮氨酸分解代谢途径的证明。
Biochem J. 1976 Dec 15;160(3):417-26. doi: 10.1042/bj1600417a.
引用本文的文献
1
Severe child form of primary hyperoxaluria type 2 - a case report revealing consequence of GRHPR deficiency on metabolism.2型原发性高草酸尿症的严重儿童形式——一例揭示GRHPR缺乏对代谢影响的病例报告。
BMC Med Genet. 2017 May 31;18(1):59. doi: 10.1186/s12881-017-0421-8.
2
Gaussian and linear deconvolution of LC-MS/MS chromatograms of the eight aminobutyric acid isomers.八种氨基丁酸异构体的液相色谱-串联质谱色谱图的高斯和线性去卷积
Anal Biochem. 2017 Jan 1;516:75-85. doi: 10.1016/j.ab.2016.10.017. Epub 2016 Oct 19.
3
New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid?二氢嘧啶脱氢酶缺乏症的新见解：β-氨基异丁酸起关键作用？
Biochem J. 2004 Apr 1;379(Pt 1):119-24. doi: 10.1042/BJ20031463.
本文引用的文献
1
The purification and properties of beta-hydroxyisobutyric dehydrogenase.β-羟基异丁酸脱氢酶的纯化及性质
J Biol Chem. 1957 Mar;225(1):511-21.
2
3
Linear relationship between the R- and S-enantiomers of a beta-aminoisobutyric acid in human urine.人尿中β-氨基异丁酸R-和S-对映体之间的线性关系。
Clin Chim Acta. 1981 Nov 11;116(3):261-7. doi: 10.1016/0009-8981(81)90045-0.
4
Metabolism of malonic semialdehyde in man.人体内丙二酸半醛的代谢
Biochem J. 1983 Oct 15;216(1):81-5. doi: 10.1042/bj2160081.
5
D-beta-aminoisobutyrate:pyruvate aminotransferase in mammalian liver and excretion of beta-aminoisobutyrate by man.D-β-氨基异丁酸：丙酮酸转氨酶在哺乳动物肝脏中的作用及人体β-氨基异丁酸的排泄
J Biol Chem. 1969 Jan 25;244(2):335-40.
6
Properties of purified methylmalonate semialdehyde dehydrogenase of Pseudomonas aeruginosa.铜绿假单胞菌纯化的甲基丙二酸半醛脱氢酶的特性
J Biol Chem. 1970 Apr 10;245(7):1828-35.
7
Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.β-丙氨酸、3-羟基丙酸、R-和S-3-氨基异丁酸、R-和S-3-羟基异丁酸以及S-2-(羟甲基)丁酸排泄过多，可能是由于相应丙二酸半醛代谢缺陷所致。
J Inherit Metab Dis. 1985;8(2):75-9. doi: 10.1007/BF01801669.
8
[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.利用核磁共振研究甲基丙二酸血症中[13C]缬氨酸代谢：丙酸酯作为必需中间体
Proc Natl Acad Sci U S A. 1975 Sep;72(9):3692-6. doi: 10.1073/pnas.72.9.3692.
9
10
Isolation and identification of S(+)-3-hydroxyisobutyric acid in the urine of rats loaded with isobutyric acid.异丁酸负荷大鼠尿液中S(+)-3-羟基异丁酸的分离与鉴定
Biochim Biophys Acta. 1979 Jul 18;585(4):643-4. doi: 10.1016/0304-4165(79)90196-x.
Zotero 插件