Document Detail

Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism.
MedLine Citation:
PMID:  2919129     Owner:  NLM     Status:  MEDLINE    
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.
U Wendel
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric research     Volume:  25     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  1989 Feb 
Date Detail:
Created Date:  1989-04-06     Completed Date:  1989-04-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  147-50     Citation Subset:  IM    
Kinderklinik, Universität Düsseldorf, Federal Republic of Germany.
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MeSH Terms
Child, Preschool
Erythrocyte Membrane / analysis*
Fatty Acids / analysis*
Infant, Newborn
Malonates / urine*
Membrane Lipids / analysis*
Metabolism, Inborn Errors / blood*
Methylmalonic Acid / urine*
Phosphatidylcholines / analysis
Phosphatidylethanolamines / analysis
Propionates / blood*
Propionic Acids / blood*,  metabolism
Reg. No./Substance:
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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