Linked Life Data

9694508

Source:http://linkedlifedata.com/resource/pubmed/id/9694508

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
1998-8-18
pubmed:abstractText
Congenital dyserythropoietic anemia type II (CDA-II) is the most common form of inherited dyserythropoiesis. Erythroid precursor and red blood cells (RBCs) show characteristic morphological abnormalities. Biochemical studies have shown that this disease is associated with reduced glycosylation activity, which endows band 3 (anion transporter) with peculiar characteristics. The life span of RBCs may be shortened in patients with CDA-II, a phenomenon that has been ascribed to this membrane defect. We analyzed seven unrelated patients with CDA-II and five control subjects. In all of the CDA-II patients, erythrocytes presented a band 3 that was thinner than usual and also migrated slightly faster on SDS-PAGE. Analysis of anion transport function in CDA-II RBC samples demonstrated decreased anion exchange activity per band 3 molecule. Furthermore, we observed that the CDA-II RBCs contained larger amounts of aggregate band 3 than control erythrocytes. Aggregate band 3 has been reported to bind naturally occurring antibodies that mediate the phagocytic removal of RBCs. We provide evidence that both the phagocytic index (RBCs/macrophage) and the amount of membrane-bound immunoglobulin (IgG) are elevated in CDA-II erythrocytes. Our results suggest that the mild hemolysis observed in patients with CDA-II may be ascribed to clusterization of band 3, which leads to IgG binding and phagocytosis, and not to a secondary modification of the cytoskeletal structure of RBCs.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0301-472X
pubmed:author
pubmed:issnType
Print
pubmed:volume
26
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
869-73
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1998
pubmed:articleTitle
Decreased band 3 anion transport activity and band 3 clusterization in congenital dyserythropoietic anemia type II.
pubmed:affiliation
Department of Internal Medicine, University of Verona, Italy.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't