Primary immunodeficiency disorder (PID) refers to a large heterogeneous group of disorders that result from defects in immune system development and/or function.
Christine McCusker +2 more
doaj +1 more source
From mice to humans—divergent strategies for intestinal homeostasis and regeneration
Recent advances such as organoid genome editing, xenotransplantation, imaging, and whole‐genome sequencing have enabled direct studies of human intestinal stem cells (ISCs). These studies reveal species‐specific features, including slower ISC proliferation, distinct injury responses, slower somatic mutation accumulation in humans, and an inverse ...
Keiko Ishikawa +2 more
wiley +1 more source
Liver organoids: modelling complexity in homeostasis and disease
Studying liver in vitro has been challenging because simple 2D cell cultures fail to capture liver's cellular and architectural complexity. To bridge this gap, scientists increasingly use organoids, 3D liver models which better mimic liver composition and function. This review examines recent advances in liver organoid complexity and realism, discusses
Anna M. Dowbaj, Meritxell Huch
wiley +1 more source
Clinical features and mutational analysis of X-linked agammaglobulinemia patients in Malaysia
BackgroundBruton’s tyrosine kinase (BTK) is a cytoplasmic protein involved in the B cell development. X-linked agammaglobulinemia (XLA) is caused by mutation in the BTK gene, which results in very low or absent B cells.
Chai Teng Chear +17 more
doaj +1 more source
Small intestine involvement in primary immunodeficiency
Common variable immunodeficiency is the most frequent among all primary immunodeficiencies and is characterized by decrease of immunoglobulin secretion which causes the extent of clinical presentation of the disease.
A V Chuklyaeva, E G Borkhanova
core +1 more source
The ubiquitin system in normal and infected germinal center B cells
The ubiquitin system plays a central role in germinal center (GC) B cells, influencing differentiation to long‐lived memory B cells. Oncogenic gammaherpesviruses gain access to memory B cells by establishing latency in GC B cells. Mapping ubiquitin mechanisms in normal and infected GC B cells will define specific molecular circuits in B cells germane ...
Destiny Davis +2 more
wiley +1 more source
Ureaplasma parvum septic arthritis in LRBA deficiency: An infectious mimic of autoimmune arthritisUreaplasma parvum septic arthritis in LRBA deficiency [PDF]
Timmermans et al. report culture-negative septic arthritis caused by Ureaplasma parvum in a patient with LRBA deficiency, mimicking autoimmune disease.
Katrien Timmermans +8 more
doaj +1 more source
Diminished toll-like receptor response in febrile infection-related epilepsy syndrome (FIRES)
Background: Defective human TLR3 signaling causes recurrent and refractory herpes simplex encephalitis/encephalopathy. Children with febrile infection-related epilepsy syndrome with refractory seizures may have defective TLR responses.
Meng-Ying Hsieh +6 more
doaj +1 more source
Stem cell transplantation for primary immunodeficiency diseases
The formation of the Primary Immune Deficiency Treatment Consortium, a multiinstitutional North American consortium, has contributed to our understanding of outcomes after transplant for primary ...
Cowan, MJ +5 more
core +1 more source
Going viral − Virus‐targeting nanobodies for research, diagnostics and therapy
The increasing prevalence of viral diseases represents a major threat for global human health. Nanobodies, small binding fragments derived from camelid heavy‐chain antibodies, offer versatile tools for viral research, diagnostics, and antiviral therapy.
Domenic Ebert, Ulrich Rothbauer
wiley +1 more source

