Literature DB >> 2575466

Assay of methylmalonyl CoA mutase with high-performance liquid chromatography.

M Kikuchi1, H Hanamizu, K Narisawa, K Tada.   

Abstract

An assay for methylmalonyl CoA mutase activity is described. Succinyl CoA produced in this method is separated from the substrate, methylmalonyl CoA, by reverse-phase high-performance liquid chromatography and is quantified. This method is useful to differentiate mutase apoenzyme deficiency (mut0, mut-) and the defect in deoxyadenosylcobalamin synthesis using fibroblasts cultured in high concentration of supplementary hydroxocobalamin. In methylmalonic acidemia, measurement of lymphocytes mutase activity offers therapeutical and prognostic informations.

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Year:  1989        PMID： 2575466     DOI： 10.1016/0009-8981(89)90064-8

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  7 in total

1.  Mutation Profile of the MUT Gene in Chinese Methylmalonic Aciduria Patients.

Authors:  Mei-Ying Liu; Tze-Tze Liu; Yang-Ling Yang; Ying-Chen Chang; Ya-Ling Fan; Shu-Fen Lee; Yu-Ting Teng; Szu-Hui Chiang; Dau-Ming Niu; Shio-Jean Lin; Mei-Chun Chao; Shuan-Pei Lin; Lian-Shu Han; Yu Qi; Kwang-Jen Hsiao
Journal:  JIMD Rep       Date:  2012-01-31

2.  Correction of methylmalonic aciduria in vivo using a codon-optimized lentiviral vector.

Authors:  Edward S Y Wong; Chantelle McIntyre; Heidi L Peters; Enzo Ranieri; Donald S Anson; Janice M Fletcher
Journal:  Hum Gene Ther       Date:  2014-04-02       Impact factor: 5.695

3.  Methylmalonic Acidemia Diagnosis by Laboratory Methods.

Authors:  Fatemeh Keyfi; Saeed Talebi; Abdol-Reza Varasteh
Journal:  Rep Biochem Mol Biol       Date:  2016-10

4.  Eight novel MUT loss-of-function missense mutations in Chinese patients with isolated methylmalonic academia.

Authors:  Lian-Shu Han; Zhuo Huang; Feng Han; Yu Wang; Zhu-Wen Gong; Xue-Fan Gu
Journal:  World J Pediatr       Date:  2017-01-19       Impact factor: 2.764

5.  Towards metabolic sink therapy for mut methylmalonic acidaemia: correction of methylmalonyl-CoA mutase deficiency in T lymphocytes from a mut methylmalonic acidaemia child by retroviral-mediated gene transfer.

Authors:  C C Chang; K J Hsiao; Y M Lee; C M Lin
Journal:  J Inherit Metab Dis       Date:  1999-10       Impact factor: 4.982

6.  Mutation and haplotype analyses of the MUT gene in Japanese patients with methylmalonic acidemia.

Authors:  Osamu Sakamoto; Toshihiro Ohura; Yoichi Matsubara; Masaki Takayanagi; Shigeru Tsuchiya
Journal:  J Hum Genet       Date:  2006-10-31       Impact factor: 3.172

7.  Optimization and validation of a reversed-phase high performance liquid chromatography method for the measurement of bovine liver methylmalonyl-coenzyme a mutase activity.

Authors:  Bazoumana Ouattara; Mélissa Duplessis; Christiane L Girard
Journal:  BMC Biochem       Date:  2013-10-16       Impact factor: 4.059
  7 in total

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