Abatacept as a long-term targeted therapy for LRBA deficiency

A Kiykim, I Ogulur, E Dursun, LM Charbonnier… - The Journal of Allergy …, 2019 - Elsevier
Background LPS-responsive beige-like anchor (LRBA) deficiency presents with
susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of …

Autoimmunity and primary immunodeficiency: two sides of the same coin?

RE Schmidt, B Grimbacher, T Witte - Nature Reviews Rheumatology, 2018 - nature.com
Autoimmunity and immunodeficiency were previously considered to be mutually exclusive
conditions; however, increased understanding of the complex immune regulatory and …

Translating immunology into therapeutic concepts for inflammatory bowel disease

HH Uhlig, F Powrie - Annual review of immunology, 2018 - annualreviews.org
Inflammatory bowel disease (IBD) defines a spectrum of complex disorders. Understanding
how environmental risk factors, alterations of the intestinal microbiota, and polygenetic and …

[HTML][HTML] Prevalence of immune-related systemic adverse events in patients treated with anti-Programmed cell Death 1/anti-Programmed cell Death-Ligand 1 agents: A …

S Le Burel, S Champiat, C Mateus, A Marabelle… - European Journal of …, 2017 - Elsevier
Aim The growing use of immune checkpoint inhibitors (ICIs) is associated with the
occurrence of immune-related adverse events (irAEs). Few data are published on systemic …

[PDF][PDF] Regulatory T cells in tumor microenvironment and approach for anticancer immunotherapy

JH Kim, BS Kim, SK Lee - Immune network, 2020 - synapse.koreamed.org
Tregs have a role in immunological tolerance and immune homeostasis by suppressing
immune reactions, and its therapeutic potential is critical in autoimmune diseases and …

Haploinsufficiency of the NF-κB1 subunit p50 in common variable immunodeficiency

M Fliegauf, VL Bryant, N Frede, C Slade… - The American Journal of …, 2015 - cell.com
Common variable immunodeficiency (CVID), characterized by recurrent infections, is the
most prevalent symptomatic antibody deficiency. In∼ 90% of CVID-affected individuals, no …

Spectrum of Phenotypes Associated with Mutations in LRBA

OK Alkhairy, H Abolhassani, N Rezaei, M Fang… - Journal of clinical …, 2016 - Springer
To date, several germline mutations have been identified in the LRBA gene in patients
suffering from a variety of clinical symptoms. These mutations abolish the expression of the …

Tregopathies: monogenic diseases resulting in regulatory T-cell deficiency

AM Cepika, Y Sato, JMH Liu, MJ Uyeda… - Journal of Allergy and …, 2018 - Elsevier
Monogenic diseases of the immune system, also known as inborn errors of immunity, are
caused by single-gene mutations resulting in immune deficiency and dysregulation. More …

CTLA-4–mediated transendocytosis of costimulatory molecules primarily targets migratory dendritic cells

V Ovcinnikovs, EM Ross, L Petersone, NM Edner… - Science …, 2019 - science.org
CTLA-4 is a critical negative regulator of the immune system and a major target for
immunotherapy. However, precisely how it functions in vivo to maintain immune …

[HTML][HTML] A long-lived IL-2 mutein that selectively activates and expands regulatory T cells as a therapy for autoimmune disease

LB Peterson, CJM Bell, SK Howlett, ML Pekalski… - Journal of …, 2018 - Elsevier
Susceptibility to multiple autoimmune diseases is associated with common gene
polymorphisms influencing IL-2 signaling and T reg function, making T reg-specific …