Document Detail

Decrease of palmitoyl-CoA elongation in platelets and leukocytes in the patient of hereditary methemoglobinemia associated with mental retardation.
MedLine Citation:
PMID:  3675585     Owner:  NLM     Status:  MEDLINE    
Effect of the deficiency of NADH-cytochrome b5 reductase on fatty acid elongation was studied in the platelets and leukocytes taken from a patient of hereditary methemoglobinemia associated with mental retardation. The activity of fatty acid elongation was determined by measuring the incorporation of [2-14C]malonyl-CoA into palmitoyl-CoA. The de novo biosynthesis of fatty acids was blocked by the addition of phosphotransacetylase, and the elongation system could be assayed in the homogenates separated from de novo biosynthesis. As compared to normal subjects approximately 40% decrease of fatty acid elongation was observed both in the platelets and leukocytes from the patient.
M Takeshita; M Tamura; M Kugi; T Matsuki; Y Yoneyama; T Igarashi
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Publication Detail:
Type:  In Vitro; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Biochemical and biophysical research communications     Volume:  148     ISSN:  0006-291X     ISO Abbreviation:  Biochem. Biophys. Res. Commun.     Publication Date:  1987 Oct 
Date Detail:
Created Date:  1987-12-01     Completed Date:  1987-12-01     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372516     Medline TA:  Biochem Biophys Res Commun     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  384-91     Citation Subset:  IM    
Department of Biochemistry, Medical College of Oita, Japan.
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MeSH Terms
Acyl Coenzyme A / blood*
Blood Platelets / metabolism*
Leukocytes / metabolism*
Malonyl Coenzyme A / blood
Mental Retardation / blood*,  etiology
Methemoglobinemia / blood*,  genetics,  psychology
Palmitoyl Coenzyme A / blood*
Reg. No./Substance:
0/Acyl Coenzyme A; 1763-10-6/Palmitoyl Coenzyme A; 524-14-1/Malonyl Coenzyme A

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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