Document Detail

Adrenoleukodystrophy. The chain shortening of erucic acid (22:1(n-9)) and adrenic acid (22:4(n-6)) is deficient in neonatal adrenoleukodystrophy and normal in X-linked adrenoleukodistrophy skin fibroblasts.
MedLine Citation:
PMID:  2538146     Owner:  NLM     Status:  MEDLINE    
The metabolism of long chain unsaturated fatty acids was studied in cultured fibroblasts from patients with X-linked adrenoleukodystrophy (ALD) and with neonatal ALD. By using [14-14C] erucic acid (22:1(n-9)) as substrate it was shown that the peroxisomal beta-oxidation, measured as chain shortening, was impaired in cells from patients with neonatal ALD. The beta-oxidation of adrenic acid (22:4(n-6)), measured as acid-soluble products, was also reduced in the neonatal ALD cells. The peroxisomal beta-oxidation of [14-14C]erucic acid (22:1(n-9)) and [2-14C]adrenic acid (22:4(n-6)) was normal in cells from X-ALD patients. The beta-oxidation, esterification and chain elongation of [1-14C]arachidonic acid (20:4(n-6)) and [1-14C]eicosapentaenoic acid (20:5(n-3)) was normal in both X-linked ALD and in neonatal ALD. Previous studies suggest that the activation of very long chain fatty acids by a lignoceryl (24:0)-CoA ligase is deficient in X-linked ALD, while the peroxisomal beta-oxidation enzymes are deficient in neonatal ALD. The present results suggest that the peroxisomal very long-chain acyl-CoA ligase is not required for activation of unsaturated C20 and C22 fatty acids and that these fatty acids can be efficiently activated by the long chain acyl-(palmityl)-CoA ligase.
E Christensen; M Grønn; T A Hagve; B F Kase; B O Christophersen
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Biochimica et biophysica acta     Volume:  1002     ISSN:  0006-3002     ISO Abbreviation:  Biochim. Biophys. Acta     Publication Date:  1989 Mar 
Date Detail:
Created Date:  1989-04-28     Completed Date:  1989-04-28     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0217513     Medline TA:  Biochim Biophys Acta     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  79-83     Citation Subset:  IM    
Institute of Clinical Biochemistry, University of Oslo, Norway.
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MeSH Terms
Adrenoleukodystrophy / genetics,  metabolism*
Arachidonic Acid
Arachidonic Acids / metabolism
Cells, Cultured
Chondrodysplasia Punctata / metabolism
Diffuse Cerebral Sclerosis of Schilder / metabolism*
Eicosapentaenoic Acid / metabolism
Erucic Acids / metabolism*
Fatty Acids, Monounsaturated / metabolism*
Fibroblasts / metabolism*
Infant, Newborn
Microbodies / metabolism
X Chromosome*
Reg. No./Substance:
0/Arachidonic Acids; 0/Erucic Acids; 0/Fatty Acids, Monounsaturated; 1072-39-5/erucic acid; 1553-41-9/Eicosapentaenoic Acid; 2091-25-0/adrenic acid; 506-32-1/Arachidonic Acid

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