Common variable immunodeficiency associated with myelocathexis and altered membrane sodium-proton antiport.
Alterations in protein kinase C (PKC) activity have been implied in the pathogenesis of common variable immunodeficiency (CVID). We analyzed amiloride-sensitive red blood cell Na+/H+ exchange (sodium-proton antiport, SPA) and its response to protein kinase stimulation in a patient with CVID. Compared with healthy subjects or patients with sepsis, a unique pattern of SPA activation has been shown. The patient's SPA was decreased and unresponsive to PKC stimulation, whereas stimulation by insulin, a tyrosine kinase activator, restored SPA activity. An alteration of serine-threonine phosphorylation is suggested as a possible mechanism for the immune failure.
Gur
H
H
Department of Medicine C, Chaim Sheba Medical Center Tel Hashomer, Sackler, Faculty of Medicine, Tel Aviv University, Israel.
Koldanov
R
R
Segal
A
A
Schiby
G
G
Spielberg
O
O
Koren
W
W
D000328:Adult; D000917:Antibody Formation; D001853:Bone Marrow / Q000473:pathology; D017074:Common Variable Immunodeficiency / Q000378:metabolism* / Q000473:pathology; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006801:Humans; D010766:Phosphorylation; D011493:Protein Kinase C / Q000378:metabolism; D017923:Sodium-Hydrogen Exchangers / Q000097:blood*
D017923:Sodium-Hydrogen Exchangers; D011493:Protein Kinase C