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

Abstract:

: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.

journal_name

Blood

journal_title

Blood

authors

Telen MJ,Green AM

subject

Has Abstract

pub_date

1989-07-01 00:00:00

pages

437-41

issue

1

eissn

0006-4971

issn

1528-0020

journal_volume

74

pub_type

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