Primary immunodeficiency disorders articles from across Nature Portfolio

Loss-of-function mutations in the chromatin remodelling protein HELLS result in humoral immune deficiency. Authors here show in a conditional knockout mouse model that HELLS controls the kinetics of a typical germinal center response by DNA methylation, its absence leading to either the appearance of memory-B cell markers or a metabolic state change typical of plasma cells.

Modeling a rare bone marrow failure disorder due to haploinsufficiency for the MECOM transcription factor identifies a human hematopoietic stem cell regulatory network, which is co-opted by high-risk leukemias.

Long-term follow-up data reinforce the curative potential of hematopoietic stem-cell gene therapy for this rare primary immunodeficiency disorder.

To cope with the new situation during the pandemic, the Turkish people have united in the fight against SARS-CoV-2 with their healthcare workers, scientists and government. We are waiting for the global pandemic to end soon.

A new study shows successful immunotherapy of a detrimental gastrointestinal disease using the complement inhibitor eculizumab.

PI3Kδ regulates adaptive immune thresholds and limits immunity to endogenous microbiota.

In the current issue of Nature Immunology, Casanova and colleagues demonstrate that humans (and mouse models) with autosomal-recessive SPPL2a deficiency have a severe defect in conventional dendritic cell 2 survival and production of IL-12 and IL-23, and diminished IFN-γ secretion by mycobacterium-specific memory T cells, thus resulting in increased susceptibility to mycobacterial diseases.