Inborn errors of human B cell development, differentiation, and function

SG Tangye, T Nguyen, EK Deenick, VL Bryant… - Journal of Experimental …, 2023 - rupress.org
B cells develop from hematopoietic stem cells in the bone marrow. Once generated, they
serve multiple roles in immune regulation and host defense. However, their most important …

Common variable immunodeficiency: more pathways than roads to Rome

XP Peng, A Caballero-Oteyza… - Annual Review of …, 2023 - annualreviews.org
Fifty years have elapsed since the term common variable immunodeficiency (CVID) was
introduced to accommodate the many and varied antibody deficiencies being identified in …

Hepatocellular cystathionine γ lyase/hydrogen sulfide attenuates nonalcoholic fatty liver disease by activating farnesoid X receptor

W Xu, C Cui, C Cui, Z Chen, H Zhang, Q Cui, G Xu… - Hepatology, 2022 - journals.lww.com
Inborn errors of immunity (IEIs) consist of numerous rare, inherited defects of the immune
system that affect about 500,000 people in the United States. As advancements in diagnosis …

Cellular and molecular mechanisms breaking immune tolerance in inborn errors of immunity

G Sogkas, F Atschekzei, IR Adriawan… - Cellular & Molecular …, 2021 - nature.com
In addition to susceptibility to infections, conventional primary immunodeficiency disorders
(PIDs) and inborn errors of immunity (IEI) can cause immune dysregulation, manifesting as …