Document Detail

Contribution of odd-numbered fatty acid oxidation to propionate production in neonates with methylmalonic and propionic acidaemias.
MedLine Citation:
PMID:  8131803     Owner:  NLM     Status:  MEDLINE    
Newborns with disturbed propionate metabolism (propionic and methylmalonic acidaemias) accumulate during fetal life large amounts of odd-numbered long-chain fatty acids in adipose tissue (8% to 10% of total fatty acids vs 1% in normal controls) and other body lipids. During periods of acute catabolism, such as in the early days of life, mobilization and oxidation of odd-chain fatty acids from adipose tissue yield extensive amounts of toxic propionyl-CoA in the mitochondria and thus might contribute to the severe illness of the patients. Our data suggest that not only catabolism of protein but also that of adipose tissue has to be avoided in order to alleviate the neonatal illness of patients with propionic and methylmalonic acidaemias. It seems prudent to avoid prolonged fasting during the 1st year of life.
U Wendel; R Zass; D Leupold
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European journal of pediatrics     Volume:  152     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  1993 Dec 
Date Detail:
Created Date:  1994-04-19     Completed Date:  1994-04-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  1021-3     Citation Subset:  IM    
Children's Hospital, Heinrich-Heine Universität Düsseldorf, Germany.
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MeSH Terms
Acyl Coenzyme A / metabolism
Adipose Tissue / metabolism
Amino Acid Metabolism, Inborn Errors / metabolism*
Fatty Acids / metabolism*
Infant, Newborn
Methylmalonic Acid / blood*
Mitochondria / metabolism
Propionates / metabolism*
Propionic Acids / blood*
Reg. No./Substance:
0/Acyl Coenzyme A; 0/Fatty Acids; 0/Propionates; 0/Propionic Acids; 317-66-8/propionyl-coenzyme A; 516-05-2/Methylmalonic Acid

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