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Predicate | Object |
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rdf:type | |
lifeskim:mentions |
umls-concept:C0003695,
umls-concept:C0016030,
umls-concept:C0030705,
umls-concept:C0205349,
umls-concept:C0220781,
umls-concept:C0556150,
umls-concept:C0681850,
umls-concept:C1123023,
umls-concept:C1550501,
umls-concept:C1706203,
umls-concept:C1883254,
umls-concept:C2349001,
umls-concept:C2587213,
umls-concept:C2697811
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pubmed:issue |
3
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pubmed:dateCreated |
1999-5-19
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pubmed:abstractText |
The beta-oxidation of [3H] arachidonic acid (AA; 20:4 n-6) and the conversion of [1-14C]eicosapentaenoic acid (EPA, 20:5 n-3) to docosahexaenoic acid (DHA, 22:6 n-3) have been studied in skin fibroblasts from patients with inherited peroxisomal diseases, such as Zellweger (ZW) and X-linked adrenoleukodystrophy (X-ALD), from patients with Alzheimer's disease (AD), a non-inherited neuropathology, and from controls. EPA is not converted to DHA, while there is enhanced formation of the intermediate product 22:5 n-3 in ZW, when compared to X-ALD, AD and controls. We also confirmed that AA is not beta-oxidized to 4,7,10-hexadecatrienoic acid (16:3), a metabolite produced by peroxisomes, while being more effectively converted to the elongation product 22:4, in ZW, in comparison to X-ALD, AD and controls. The data demonstrate a defect in DHA synthesis and in AA beta-oxidation, and the occurrence of associated adaptative modifications in the metabolism of these long chain PUFA, in three Italian ZW patients.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
0304-3940
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
10
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pubmed:volume |
250
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
145-8
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:9708853-Adrenoleukodystrophy,
pubmed-meshheading:9708853-Alzheimer Disease,
pubmed-meshheading:9708853-Arachidonic Acid,
pubmed-meshheading:9708853-Docosahexaenoic Acids,
pubmed-meshheading:9708853-Fibroblasts,
pubmed-meshheading:9708853-Humans,
pubmed-meshheading:9708853-Oxidation-Reduction,
pubmed-meshheading:9708853-Skin,
pubmed-meshheading:9708853-Zellweger Syndrome
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pubmed:year |
1998
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pubmed:articleTitle |
The beta-oxidation of arachidonic acid and the synthesis of docosahexaenoic acid are selectively and consistently altered in skin fibroblasts from three Zellweger patients versus X-adrenoleukodystrophy, Alzheimer and control subjects.
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pubmed:affiliation |
Institute of Pharmacological Sciences, University of Milan, Italy. petroni@imiucca.csi.unimi.it
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pubmed:publicationType |
Journal Article,
Comparative Study
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