Agammaglobulinemia with normal B-cell numbers in a patient lacking Bob1.

Patrick Kury,Julian Staniek,Oliver Wegehaupt,Iga Janowska,Matthias Eckenweiler,Rudolf Korinthenberg,Natia Japaridze,Manuela Pendziwiat,Ingo Helbig,Els Verhoeyen,Johannes Jung,Andres Caballero Garcia de Oteyza,Michele Proietti,Tamar Phirtskhalaishvili,Irakli Rtskhiladze,Peter J. Nielsen,Stephan Ehl,Carsten Speckmann,Marta Rizzi

Agammaglobulinemia with normal B-cell numbers in a patient lacking Bob1.

2021

Patrick Kury
Julian Staniek
Oliver Wegehaupt
Iga Janowska
Matthias Eckenweiler
Rudolf Korinthenberg
Natia Japaridze
Manuela Pendziwiat
Ingo Helbig
Els Verhoeyen
Johannes Jung
Andres Caballero Garcia de Oteyza
Michele Proietti
Tamar Phirtskhalaishvili
Irakli Rtskhiladze
Peter J. Nielsen
Stephan Ehl
Carsten Speckmann
Marta Rizzi

CAPSULE SUMMARY Bob1 deficiency reduces B-cell responsiveness, impairs plasmablast formation and immunoglobulin secretion. Bob1 deficiency presents with increased susceptibility to respiratory infections and agammaglobulinemia despite normal B-cell numbers.

Keywords:

Antibody
Immunodeficiency
Immunology
Respiratory system
Medicine
B-cell receptor
Capsule
Normal B cell numbers
Immunoglobulin secretion

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