Linked Life Data

18302715

Source:http://linkedlifedata.com/resource/pubmed/id/18302715

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2008-2-27
pubmed:abstractText
beta-Thalassaemia is a congenital haemoglobinopathy, associated with red blood cells (RBC) anomalies, leading to impairment of their flow-affecting properties, namely, RBC deformability, self-aggregability, and adherence to endothelial cells (EC). Treatment of normal RBC with phenylhydrazine (PHZ) causes selective association of oxidized alpha-globin chains with the membrane skeleton, leading to reduced RBC deformability, characteristic of beta-thalassaemia. PHZ has thus been used to mimic phenotypes of beta-thalassaemia RBC. The present study was undertaken to further elucidate the suitability of PHZ-treated RBC as a model for beta-thalassemic RBC, by comparing the aggregability and adhesiveness of PHZ-treated RBC to those of RBC from thalassaemia intermedia (TI) patients, using image analysis of RBC under flow. In addition, the externalization of phosphatidylserine (PS), a mediator of RBC/EC interaction, was determined. It was found that PHZ caused enhanced RBC adhesiveness to extracellular matrix, similar to TI-RBC. Furthermore, in both conditions, the enhanced adhesiveness was mediated by PS translocated to the RBC surface. In contrast, PHZ treatment completely abolished RBC aggregability, while TI-RBC aggregability was slightly elevated. It is proposed that PHZ-treated RBC resemble beta-thalassaemia RBC in their deformability and adhesiveness, but not in their aggregability, and thus can be used as a limited model for beta-thalassaemia RBC phenotypes.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
1365-2141
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
140
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
692-700
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
Phenylhydrazine as a partial model for beta-thalassaemia red blood cell hemodynamic properties.
pubmed:affiliation
Department of Biochemistry, Hebrew University-Hadassah Medical School, Jerusalem, Israel.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't