The Inab phenotype: characterization of the membrane protein and complement regulatory defect.

Published

Journal Article

Recent demonstration that Cromer-related human blood group antigens reside on decay-accelerating factor (DAF) has led to identification of an apparent null phenotype (Inab) for erythrocyte DAF. This study examined expression of other phosphatidylinositol (PI)-anchored proteins by Inab erythrocytes and showed that the PI-linked membrane proteins acetylcholinesterase (AchE) and lymphocyte function-associated antigen-3 (LFA-3) are normally expressed by these cells. Furthermore, studies of the complement sensitivity of Inab RBCs demonstrated them to be abnormally complement sensitive, with an apparent defect in downregulation of C3 convertase activity. Thus, the Inab phenotype appears to represent an instance of hereditary erythrocyte DAF deficiency whose mechanism differs from that of paroxysmal nocturnal hemoglobinuria (PNH) and which is unassociated with clinically evident hemolytic disease.

Full Text

Duke Authors

Cited Authors

  • Telen, MJ; Green, AM

Published Date

  • July 1, 1989

Published In

Volume / Issue

  • 74 / 1

Start / End Page

  • 437 - 441

PubMed ID

  • 2473800

Pubmed Central ID

  • 2473800

International Standard Serial Number (ISSN)

  • 0006-4971

Language

  • eng

Conference Location

  • United States