TY - JOUR
T1 - Defective self-reactive antibody repertoire of serum IgM in patients with hyper-IgM syndrome
AU - Lacroix-Desmazes, Sébastien
AU - Resnick, Igor
AU - Stahl, Dorothea
AU - Mouthon, Luc
AU - Espanol, Teresa
AU - Levy, Jacov
AU - Kaveri, Srini V.
AU - Notarangelo, Luigi
AU - Eibl, Martha
AU - Fischer, Alain
AU - Ochs, Hans
AU - Kazatchkine, Michel D.
PY - 1999/5/1
Y1 - 1999/5/1
N2 - We have analyzed the self-reactive repertoires of IgM and IgG Abs in the serum of 19 patients with hyper-IgM syndrome (HIM) by means of a quantitative immunoblotting technique that allows for a quantitative comparison of Ab repertoires in health and disease by multiparametric statistical analysis. Normal tissue extracts of liver, lung, stomach, and kidney were used as sources of self Ags. Extracts of Pseudomonas aeruginosa and Staphylococcus epidermidis were used as sources of nonself Ags. We demonstrate a significant bias in repertoires of reactivities of IgM of patients with HIM with self Ags. Ab repertoires of IgM toward nonself Ags did not differ, however, between patients and controls. No difference was found between IgM repertoires of untreated patients and those of patients receiving substitutive treatment with i.v. IgG. IgG in the serum of HIM patients lacked reactivity with self Ags, although it exhibited a pattern of reactivity with nonself Ags that was similar to that of IgG of healthy controls. The data demonstrate that functional CD40-CD40 ligand interactions are essential for the selection of natural self-reactive B cell repertoires.
AB - We have analyzed the self-reactive repertoires of IgM and IgG Abs in the serum of 19 patients with hyper-IgM syndrome (HIM) by means of a quantitative immunoblotting technique that allows for a quantitative comparison of Ab repertoires in health and disease by multiparametric statistical analysis. Normal tissue extracts of liver, lung, stomach, and kidney were used as sources of self Ags. Extracts of Pseudomonas aeruginosa and Staphylococcus epidermidis were used as sources of nonself Ags. We demonstrate a significant bias in repertoires of reactivities of IgM of patients with HIM with self Ags. Ab repertoires of IgM toward nonself Ags did not differ, however, between patients and controls. No difference was found between IgM repertoires of untreated patients and those of patients receiving substitutive treatment with i.v. IgG. IgG in the serum of HIM patients lacked reactivity with self Ags, although it exhibited a pattern of reactivity with nonself Ags that was similar to that of IgG of healthy controls. The data demonstrate that functional CD40-CD40 ligand interactions are essential for the selection of natural self-reactive B cell repertoires.
UR - http://www.scopus.com/inward/record.url?scp=0033137051&partnerID=8YFLogxK
M3 - Article
C2 - 10228043
AN - SCOPUS:0033137051
SN - 0022-1767
VL - 162
SP - 5601
EP - 5608
JO - Journal of Immunology
JF - Journal of Immunology
IS - 9
ER -