Role of Lu/BCAM glycoproteins in red cell diseases

Transfusion clinique et biologique : journal de la Société française de transfusion sanguine
W El NemerC Le Van Kim

Abstract

Lu/BCAM glycoproteins (gps) are the unique erythroid receptors of laminin alpha5 chain, a major component of the extracellular matrix. They interact with the membrane skeleton by binding directly to spectrin via the Lu/BCAM RK573-574 motif. Lu/BCAM gps are involved in abnormal sickle red blood cell (RBC) adhesion to components of the vascular wall. This adhesion is activated by the phosphorylation of the Lu/BCAM long isoform Lu in a protein kinase A-dependent manner. A similar high adhesion to laminin was also observed with RBCs from Hereditary Spherocytosis (HS) patients suffering from haemolytic anaemia subsequent to spectrin deficiencies. We investigated the molecular mechanisms responsible for the Lu/BCAM-mediated abnormal RBC adhesion to laminin in sickle cell disease (SCD) and HS. We showed that SCD patients treated with hydroxycarbamide (HC) had a diminished RBC adhesion to laminin that was associated with reduced levels of the PKA upstream effector cAMP and a severe decrease in Lu isoform phosphorylation. On the other hand, we showed that increased Lu/BCAM-mediated HS RBC adhesion to laminin was independent of Lu/BCAM phosphorylation. A cellular model expressing the RK573-574AA Lu/BCAM mutant, which is unable to bind to...Continue Reading

References

Jun 27, 1998·The Journal of Biological Chemistry·W El NemerC Le Van Kim
Feb 15, 2002·Current Opinion in Hematology·Paul S Frenette
Jul 9, 2004·British Journal of Haematology·Yolande KroviarskiCaroline Le Van Kim
Jul 29, 2004·Microcirculation : the Official Journal of the Microcirculatory Society, Inc·Robert P HebbelDhananjay Kaul
Apr 15, 2005·Transfusion Medicine Reviews·Marilyn J Telen
Mar 7, 2008·Haematologica·Marie-Hélène OdièvreJean-Pierre Cartron
May 7, 2008·Annals of Internal Medicine·Sophie LanzkronJodi B Segal
May 20, 2008·Journal of Thrombosis and Haemostasis : JTH·R F SchillingM I Traver

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Citations

Dec 3, 2014·Current Opinion in Microbiology·Gudula SchmidtKlaus Aktories
Sep 8, 2012·Current Opinion in Hematology·Belinda K SingletonDavid J Anstee
Mar 8, 2019·Cancers·Roland WindischChristian Wichmann

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