A simple and rapid enzymatic assay for the branched-chain α-ketoacid dehydrogenase complex using high-performance liquid chromatography-Reference-Cited by-同舟云学术

A simple and rapid enzymatic assay for the branched-chain α-ketoacid dehydrogenase complex using high-performance liquid chromatography

Published:2004-09 Issue:5 Volume:27 Page:633-639
ISSN:0141-8955
Container-title:Journal of Inherited Metabolic Disease
language:en
Short-container-title:J Inherit Metab Dis

Author:

Tajima G.¹,Yofune H.¹,Bahagia Febriani A. D.¹,Nishimura Y.¹,Ono H.¹,Sakura N.¹

Affiliation:

1. ; Department of Pediatrics; Hiroshima University Graduate School of Biomedical Sciences, 1-2-3 Kasumi; Minami-ku Hiroshima 734-8551 Japan E-mail

Publisher

Wiley

Subject

Genetics(clinical),Genetics

Link

http://onlinelibrary.wiley.com/wol1/doi/10.1023/B:BOLI.0000042988.31581.ed/fullpdf

Reference8 articles.

1. Detection of heterozygotes in maple syrup urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures;Chuang;Am J Hum Genet,1982

2. Puri?cation and characterization of branched-chain alpha-ketoacid dehydrogenase from bovine liver mitochondria;Danner;J Biol Chem,1979

3. Maple syrup urine disease: coenzyme function and prenatal monitoring;Elsas;Metabolism,1974

4. A rapid method for assay of branched-chain keto acid decarboxylation in cultured cells and its application to prenatal diagnosis of maple syrup urine disease;Fensom;Clin Chim Acta,1978

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