Literature DB >> 4083484

Quantitation of methylmalonic acid and other dicarboxylic acids in normal serum and urine using capillary gas chromatography-mass spectrometry.

P D Marcell, S P Stabler, E R Podell, R H Allen.   

Abstract

Methylmalonic acid, succinic acid, and other dicarboxylic acids have been extracted and partially purified from serum and urine using ether extraction and high-performance liquid chromatography. The t-butyldimethylsilyl derivatives were prepared and analyzed using capillary gas chromatography-mass spectrometry with selected ion monitoring. The addition of [methyl-2H3]methylmalonic acid and [1,4-13C2]succinic acid to the starting samples made it possible to quantitate these two dicarboxylic acids. Normal ranges for methylmalonic acid and succinic acid were determined in human and rat serum and in human urine. The utilization of other internal standards would make it possible to quantitate malonic, dimethylmalonic, ethylmalonic, methylsuccinic, glutaric, and other dicarboxylic acids.

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Year:  1985        PMID： 4083484     DOI： 10.1016/0003-2697(85)90440-3

Source DB:  PubMed          Journal:  Anal Biochem        ISSN： 0003-2697            Impact factor:   3.365


  24 in total

1.  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

2.  Liver-directed recombinant adeno-associated viral gene delivery rescues a lethal mouse model of methylmalonic acidemia and provides long-term phenotypic correction.

Authors:  Nuria Carrillo-Carrasco; Randy J Chandler; Suma Chandrasekaran; Charles P Venditti
Journal:  Hum Gene Ther       Date:  2010-09       Impact factor: 5.695

3.  Elevation of total homocysteine in the serum of patients with cobalamin or folate deficiency detected by capillary gas chromatography-mass spectrometry.

Authors:  S P Stabler; P D Marcell; E R Podell; R H Allen; D G Savage; J Lindenbaum
Journal:  J Clin Invest       Date:  1988-02       Impact factor: 14.808

4.  A detailed analysis of methylmalonic acid kinetics during hemodialysis and after combined liver/kidney transplantation in a patient with mut (0) methylmalonic acidemia.

Authors:  Hilary J Vernon; C John Sperati; Joshua D King; Andrea Poretti; Neil R Miller; Jennifer L Sloan; Andrew M Cameron; Donna Myers; Charles P Venditti; David Valle
Journal:  J Inherit Metab Dis       Date:  2014-06-25       Impact factor: 4.982

5.  Liquid-Liquid Extraction and Solid Phase Extraction for Urinary Organic Acids: A Comparative Study from a Resource Constraint Setting.

Authors:  Chandrawati Kumari; Bijo Varughese; Siddarth Ramji; Seema Kapoor
Journal:  Indian J Clin Biochem       Date:  2016-02-11

6.  Carnitine metabolism in the vitamin B-12-deficient rat.

Authors:  E P Brass; S P Stabler
Journal:  Biochem J       Date:  1988-10-01       Impact factor: 3.857

7.  Inhibition of cobalamin-dependent enzymes by cobalamin analogues in rats.

Authors:  S P Stabler; E P Brass; P D Marcell; R H Allen
Journal:  J Clin Invest       Date:  1991-04       Impact factor: 14.808

Review 8.  Psychological interventions for depression in adolescent and adult congenital heart disease.

Authors:  G Y H Lip; D A Lane; T A Millane; M H Tayebjee
Journal:  Cochrane Database Syst Rev       Date:  2003

9.  Pre-clinical efficacy and dosing of an AAV8 vector expressing human methylmalonyl-CoA mutase in a murine model of methylmalonic acidemia (MMA).

Authors:  Randy J Chandler; Charles P Venditti
Journal:  Mol Genet Metab       Date:  2012-09-25       Impact factor: 4.797

10.  Long-term rescue of a lethal murine model of methylmalonic acidemia using adeno-associated viral gene therapy.

Authors:  Randy J Chandler; Charles P Venditti
Journal:  Mol Ther       Date:  2009-10-27       Impact factor: 11.454
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