Linked Life Data

2944672

Source:http://linkedlifedata.com/resource/pubmed/id/2944672

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1986-11-14
pubmed:abstractText
The presence of a beta-oxidation system in peroxisomes has been well documented. Rather than a duplicate of the mitochondrial beta-oxidation system, peroxisomes seem specially equipped to initiate the oxidation of very-long-chain fatty acids. Thus, the accumulation of very-long-chain fatty acids in tissues and body fluids from patients with a limited (X-linked adrenoleukodystrophy) or generalized (cerebro-hepato-renal (Zellweger) syndrome, infantile Refsum disease, neonatal adrenoleukodystrophy) peroxisomal dysfunction probably results from an impairment in the peroxisomal beta-oxidation system. In order to study this, we have developed an original assay which allows measurement of the overall peroxisomal beta-oxidation activity in human liver homogenates. Compared to controls, a strong deficiency of this activity was detected in liver from Zellweger patients using palmitoyl-CoA as a substrate.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0009-8981
pubmed:author
pubmed:issnType
Print
pubmed:day
30
pubmed:volume
159
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1-10
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1986
pubmed:articleTitle
Peroxisomal beta-oxidation of palmitoyl-CoA in human liver homogenates and its deficiency in the cerebro-hepato-renal (Zellweger) syndrome.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't