Linked Life Data

3618126

Source:http://linkedlifedata.com/resource/pubmed/id/3618126

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1987-9-17
pubmed:abstractText
Neuropathological examination of three males with cerebro-hepato-renal (Zellweger) syndrome (CHRS) revealed selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus. This lipidotic alteration was visualized as perikaryal or axonal enlargements with cytoplasmic striations. With the light microscope, the striated material was birefringent and resistant to traditional lipid stains; ultrastructurally, it was composed of lipid clefts, lamellae and lamellar-lipid profiles; biochemically, the affected region contained large amounts of cholesterol esterified to very long-chain fatty acids, both saturated and monounsaturated. This metabolic lesion, though localized to specific sensory neurons, suggests that a more generalized defect in neuronal fatty acid metabolism may be operative in CHRS.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
0001-6322
pubmed:author
pubmed:issnType
Print
pubmed:volume
73
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
333-43
pubmed:dateRevised
2007-11-9
pubmed:meshHeading
pubmed:year
1987
pubmed:articleTitle
Neuronal lipidosis and neuroaxonal dystrophy in cerebro-hepato-renal (Zellweger) syndrome.
pubmed:publicationType
Journal Article, Case Reports