Linked Life Data

3146313

Source:http://linkedlifedata.com/resource/pubmed/id/3146313

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
1989-3-17
pubmed:abstractText
The ceroid-lipofuscinoses are inherited lysosomal storage diseases of children and animals characterised by a fluorescent lipopigment stored in a variety of tissues. Defects in lipid metabolism or the control of lipid peroxidation have been postulated to explain their pathogenesis but the underlying biochemical defect is still unknown. In the present study lipopigment was isolated from liver, kidney, pancreas and brain of sheep affected with ceroid-lipofuscinosis. Approximately two-thirds of the lipopigment mass was protein. Sodium dodecyl sulphate polyacrylamide gel electrophoresis showed a major polypeptide band of Mr 14,800, heterogeneous polypeptides between 5,000-9,000 Mr and a major band of Mr 3,500. These were not normal lysosomal proteins. I125 radiolabeling studies indicated that they were 47% of the pancreatic lipopigment mass, the 3,500 Mr polypeptides alone accounting for 26%. Lipopigment polypeptides were not subunits of a larger protein held together by disulphide bonds. The presence of the 3,500 Mr proteins in whole affected tissue homogenates distinguished them from homogenates of normal tissues. Lipopigment phospholipids were the same species as normal lysosomal phospholipids, including bis (monoacylglycero) phosphate, a lysosomal marker. Similarly the neutral lipids, notably dolichol, ubiquinone and dolichyl esters were typical of those in lysosomal membranes. Lipopigments contained 1-1.7% metals. Analyses of them indicated a functional lysosomal origin for the lipopigment. It was concluded that low Mr proteins are specifically stored in ovine ceroid-lipofuscinosis and that this disease is a lysosomal proteinosis.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1040-3787
pubmed:author
pubmed:issnType
Print
pubmed:volume
5
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
141-58
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1988
pubmed:articleTitle
Ovine ceroid-lipofuscinosis. I: Lipopigment composition is indicative of a lysosomal proteinosis.
pubmed:affiliation
Department of Veterinary Pathology, Massey University, Palmerston North, New Zealand.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.