| Elevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiency. | |
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MedLine Citation:
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PMID: 8345822 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Citrate synthase catalyzes the condensation of acetyl-coenzyme A (CoA) and oxaloacetic acid to form citric acid. The enzyme also catalyzes the condensation of propionyl-CoA and oxaloacetic acid with a maximal reaction velocity (Vmax) approximately 10(-4) times that of acetyl-CoA to form 2-methylcitric acid, which contains two asymmetric carbon atoms and exists as two pairs of related enantiomers designated as 2-methylcitric acid I and II. Cobalamin (Cbl) deficiency can lead to increases in intracellular levels of propionyl-CoA. To assess the magnitude of increased synthesis of 2-methylcitric acid in Cbl deficiency, we developed a new capillary gas chromatographic-mass spectrometric assay and measured 2-methylcitric acid levels in serum and cerebrospinal fluid (CSF) of normal subjects and patients with clinically confirmed Cbl deficiency. The normal range for 2-methylcitric acid level was 60 to 228 nmol/L for serum in 50 normal blood donors and 323 to 1,070 nmol/L for CSF in 19 normal subjects. In 50 patients with clinically confirmed Cbl deficiency, values for 2-methylcitric acid in serum ranged from 93 to 13,500 nmol/L; 44 (88%) had values above the normal range. In five patients with clinically confirmed Cbl deficiency, levels of the sum of 2-methylcitric acid I and II ranged from 1,370 to 16,300 nmol/L in CSF, and all five (100%) patients had levels above the normal range. We conclude that levels of 2-methylcitric acid are elevated in serum and CSF of most patients with Cbl deficiency. |
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Authors:
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R H Allen; S P Stabler; D G Savage; J Lindenbaum |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Metabolism: clinical and experimental Volume: 42 ISSN: 0026-0495 ISO Abbreviation: Metab. Clin. Exp. Publication Date: 1993 Aug |
Date Detail:
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Created Date: 1993-09-03 Completed Date: 1993-09-03 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0375267 Medline TA: Metabolism Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 978-88 Citation Subset: IM |
Affiliation:
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Department of Medicine, University of Colorado Health Sciences Center, Denver 80262. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Citrate (si)-Synthase / physiology Citrates* / blood, cerebrospinal fluid, urine Female Folic Acid Deficiency / blood, cerebrospinal fluid, urine Gas Chromatography-Mass Spectrometry Humans Kidney Diseases / blood, cerebrospinal fluid, urine Male Middle Aged Stereoisomerism Vitamin B 12 Deficiency* / blood, cerebrospinal fluid, urine |
| Grant Support | |
ID/Acronym/Agency:
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AG-09834/AG/NIA NIH HHS; DK-21365/DK/NIDDK NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Citrates; 6061-96-7/2-methylcitric acid; EC 2.3.3.1/Citrate (si)-Synthase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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