Linked Life Data

8808765

Source:http://linkedlifedata.com/resource/pubmed/id/8808765

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
1997-1-22
pubmed:abstractText
Abetalipoproteinemia (ABL) is an autosomal recessive disease characterized by the inability of the liver and intestine to secrete apolipoprotein B (apoB). Mutations in the microsomal triglyceride transfer protein (MTP) gene, but not the apoB gene, are responsible for the ABL phenotype. It is not clear how loss of MTP in ABL patients leads to a complete, but specific, block in the secretion of apoB. It is to this question that our work is directed. In cultured cells lacking MTP, translocation of apoB is completely arrested, leading to the hypothesis that apoB requires MTP in order to completely enter the lumen of the endoplasmic reticulum, the site of lipoprotein assembly. We examined this hypothesis by determining the presence in plasma of distinct N-terminal apoB peptides, produced exclusively from translocation arrested apoB, in the plasma of six ABL patients and six normal subjects. The data show that N-terminal apoB peptides are present in the plasma of six ABL patients, whereas intact apoB-100 was barely detectable. Moreover, the plasma of all six ABL patients displayed a 2000-fold increase in the amount of an 85 kDa N-terminal apoB peptide relative to apoB-100. These data provide the first in vivo data supporting the essential role that MTP plays in apoB translocation. In normal humans, varied expression of MTP may be responsible for the post-transcriptional regulation of apoB secretion.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
0022-2275
pubmed:author
pubmed:issnType
Print
pubmed:volume
37
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1309-15
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1996
pubmed:articleTitle
Translocation of apolipoprotein B across the endoplasmic reticulum is blocked in abetalipoproteinemia.
pubmed:affiliation
Mammalian Cell and Molecular Biology Laboratory, San Diego State University, CA 92182, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.