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
[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.
作者信息
Tanaka K, Armitage I M, Ramsdell H S, Hsia Y E, Lipsky S R, Rosenberg L E
出版信息
Proc Natl Acad Sci U S A. 1975 Sep;72(9):3692-6. doi: 10.1073/pnas.72.9.3692.
Abstract
[Alpha-13C]- and [alpha,beta-13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl-CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA.
摘要
将[α-13C]-缬氨酸和[α,β-13C]缬氨酸依次给予一名甲基丙二酸血症患者，以阐明缬氨酸从甲基丙二酸半醛到甲基丙二酰辅酶A的代谢途径。从多个尿液样本中分离出甲基丙二酸，进行纯化，并通过13C核磁共振波谱分析。与广泛接受的观点相反，结果明确显示，甲基丙二酸半醛在转化为甲基丙二酰辅酶A之前先脱羧生成丙酸。
相似文献
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[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.
2
Rapid prenatal and postnatal detection of inborn errors of propionate, methylmalonate, and cobalamin metabolism: a sensitive assay using cultured cells.快速产前和产后检测丙酸、甲基丙二酸和钴胺素代谢的先天性代谢缺陷：一种使用培养细胞的灵敏检测方法。
Hum Genet. 1976 Dec 15;34(3):277-83. doi: 10.1007/BF00295291.
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[Methylmalonic acidemia. Diagnosis and treatment in various patients].[甲基丙二酸血症。不同患者的诊断与治疗]
Ned Tijdschr Geneeskd. 1978 May 20;122(20):707-12.
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Possible explanation for hyperglycinaemia in propionic acidaemia and methylmalonic acidaemia: propionate and methylmalonate inhibit liver and brain mitochondrial clycine transport.丙酸血症和甲基丙二酸血症中高甘氨酸血症的可能解释：丙酸盐和甲基丙二酸盐抑制肝脏和脑线粒体甘氨酸转运。
J Inherit Metab Dis. 1980;2(4):93-9. doi: 10.1007/BF01805665.
7
Metabolism of methylmalonic acid in rats. Is methylmalonyl-coenzyme a racemase deficiency symptomatic in man?大鼠体内甲基丙二酸的代谢。甲基丙二酰辅酶A消旋酶缺乏在人类中是否有症状表现？
J Clin Invest. 1983 Dec;72(6):1937-47. doi: 10.1172/JCI111158.
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Acute neonatal nonketotic hyperglycinemia: normal propionate and methylmalonate metabolism.急性新生儿非酮症高甘氨酸血症：丙酸盐和甲基丙二酸代谢正常。
Pediatr Res. 1975 Jul;9(7):559-64. doi: 10.1203/00006450-197507000-00001.
9
[Molecular pathology of the hyperglycinemias].[高甘氨酸血症的分子病理学]
Rev Esp Fisiol. 1982;38 Suppl:199-205.
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Propionate metabolism by cultured skin fibroblasts from normal individuals and patients with methylmalonicaciduria and propionicacidemia.来自正常个体以及甲基丙二酸尿症和丙酸血症患者的培养皮肤成纤维细胞的丙酸代谢
Biochem Med. 1981 Aug;26(1):28-40. doi: 10.1016/0006-2944(81)90027-2.
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High Throughput Procedure for Comparative Analysis of In Vivo Cardiac Glucose or Amino Acids Use in Cardiovascular Pathologies and Pharmacological Treatments.用于心血管疾病和药物治疗中体内心脏葡萄糖或氨基酸利用情况比较分析的高通量方法
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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。
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Metabolism of methylmalonic acid in rats. Is methylmalonyl-coenzyme a racemase deficiency symptomatic in man?大鼠体内甲基丙二酸的代谢。甲基丙二酰辅酶A消旋酶缺乏在人类中是否有症状表现？
J Clin Invest. 1983 Dec;72(6):1937-47. doi: 10.1172/JCI111158.
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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.
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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。
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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.
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Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels.丙酰辅酶A羧化酶缺乏症，伴有异亮氨酸分解代谢各级代谢产物的溢流。
Eur J Pediatr. 1979 Jan 18;130(1):1-14. doi: 10.1007/BF00441893.
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The role of enoyl-coa hydratase in the metabolism of isoleucine by Pseudomonas putida.烯酰辅酶A水合酶在恶臭假单胞菌异亮氨酸代谢中的作用。
Arch Microbiol. 1978 Apr 27;117(1):99-108. doi: 10.1007/BF00689358.
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[Methylmalonic aciduria. Classification, diagnosis and therapy (author's transl)].[甲基丙二酸尿症。分类、诊断与治疗（作者译）]
Klin Wochenschr. 1977 Jan 15;55(2):57-63. doi: 10.1007/BF01469083.
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Methylmalonic aciduria. An inborn error leading to metabolic acidosis, long-chain ketonuria and intermittent hyperglycinemia.甲基丙二酸尿症。一种导致代谢性酸中毒、长链酮尿症和间歇性高甘氨酸血症的先天性疾病。
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Defective metabolism of vitamin B 12 in fibroblasts from children with methylmalonicaciduria.甲基丙二酸尿症患儿成纤维细胞中维生素B12代谢缺陷。
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Inherited propionyl-Coa carboxylase deficiency in "ketotic hyperglycinemia".“酮症性高甘氨酸血症”中的遗传性丙酰辅酶A羧化酶缺乏症。
J Clin Invest. 1971 Jan;50(1):127-30. doi: 10.1172/JCI106466.
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