The mechanism of loss of CR1 during maturation of erythrocytes is different between factor I deficient patients and healthy donors.
During the in vivo maturation of erythrocytes, the number of CR1 per cell decreases by approximately two-thirds in 30 days. The CR1 loss is enhanced in several diseases such as SLE, AIDS, and particularly in factor I deficiency. Microvesicles enriched in CR1 and DAF are released from erythrocytes matured in vitro, leading to the same loss of both molecules. When comparing reticulocytes and erythrocytes, CR1 and DAF were lost similarly in 15 normal individuals, suggesting that vesiculation may be at the origin of CR1 loss in vivo. However, the enhanced loss of CR1 in 3 patients with factor I deficiency was contrasted with a normal loss of DAF, raising the possibility that, in this pathological condition, CR1 might be proteolytically cleaved, leaving small CR1 fragments on the erythrocytes. To answer this question, a rabbit polyclonal antibody was raised against the cytoplasmic (tail) domain of CR1, which recognised specifically CR1 of erythrocytes and urinary vesicles on Western blots. However, no CR1 fragments could be detected on erythrocytes of the factor I deficient patients although this antibody was able to recognise CR1 fragments after treatment of normal erythrocytes or urinary vesicles with elastase. These data suggest that cell surface domains rich in CR1, but not in DAF, are specifically lost in factor I deficiency.
Miot
Sylvie
S
Department of Research, University Hospital Basel, Basel, Switzerland. smiot@uhbs.ch
Marfurt
Jutta
J
Lach-Trifilieff
Estelle
E
González-Rubio
Carolina
C
López-Trascasa
Margarita
M
Sadallah
Salima
S
Schifferli
Jürg Alfred
JA
D000347:Afibrinogenemia / Q000097:blood*; D000906:Antibodies; D018958:CD55 Antigens / Q000032:analysis / Q000378:metabolism; D016022:Case-Control Studies; D004905:Erythrocyte Aging; D004912:Erythrocytes / Q000502:physiology*; D005089:Exocytosis; D005340:Fibrinogen; D006801:Humans; D015151:Immunoblotting; D017463:Receptors, Complement 3b / Q000032:analysis / Q000276:immunology / Q000378:metabolism*; D012156:Reticulocytes
D000906:Antibodies; D018958:CD55 Antigens; D017463:Receptors, Complement 3b; D005340:Fibrinogen