| Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism. | |
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MedLine Citation:
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PMID: 2919129 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In propionic acidemia and methylmalonic aciduria, disorders of propionate catabolism, excess intracellular propionyl-CoA leads to an enhanced synthesis of odd-numbered long-chain fatty acids. Red cell membrane lipids of patients with these disorders contain pentadecanoic, heptadecanoic, and heptadecenoic acids in excess of normal levels. The odd-numbered long-chain fatty acid content may reflect individual differences in severity of these disorders as well as the degree of effective dietary control. It might be a useful long term parameter for determining the effectiveness of clinical management. |
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Authors:
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U Wendel |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric research Volume: 25 ISSN: 0031-3998 ISO Abbreviation: Pediatr. Res. Publication Date: 1989 Feb |
Date Detail:
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Created Date: 1989-04-06 Completed Date: 1989-04-06 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 147-50 Citation Subset: IM |
Affiliation:
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Kinderklinik, Universität Düsseldorf, Federal Republic of Germany. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Child Child, Preschool Erythrocyte Membrane / analysis* Fatty Acids / analysis* Humans Infant Infant, Newborn Malonates / urine* Membrane Lipids / analysis* Metabolism, Inborn Errors / blood* Methylmalonic Acid / urine* Phosphatidylcholines / analysis Phosphatidylethanolamines / analysis Propionates / blood* Propionic Acids / blood*, metabolism |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids; 0/Malonates; 0/Membrane Lipids; 0/Phosphatidylcholines; 0/Phosphatidylethanolamines; 0/Propionates; 0/Propionic Acids; 516-05-2/Methylmalonic Acid; 79-09-4/propionic acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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