"Deficiency in ELF4, X-Linked": a Monogenic Disease Entity Resembling Behçet's Syndrome and Inflammatory Bowel Disease.

Defining monogenic drivers of autoinflammatory syndromes elucidates mechanisms of disease in patients with these inborn errors of immunity and can facilitate targeted therapeutic interventions. Here, we describe a cohort of patients with a Behçet's- and inflammatory bowel disease (IBD)-like disorder termed "deficiency in ELF4, X-linked" (DEX) affecting males with loss-of-function variants in the ELF4 transcription factor gene located on the X chromosome. An international cohort of fourteen DEX patients was assessed to identify unifying clinical manifestations and diagnostic criteria as well as collate findings informing therapeutic responses.

A new tolerogenic cell RORs onto the scene.

Differentiation of microbe-specific T in the gut is directed by RORγt antigen-presenting cells.

Five Medical Education Podcasts You Need to Know.

Podcasts have become increasingly popular tools for medical education in recent years. Only requiring a computer or smart phone, podcasts are readily accessible to healthcare professionals, helping to disseminate medical information quickly and creating a wide community of listeners. With numerous medical podcasts available and limited spare time, it can be challenging for a healthcare professional to identify the most high-yield podcast.

Flow cytometric identification of T13 cells in mouse and human.

Anaphylaxis is a life-threatening allergic reaction caused by cross-linking of high-affinity IgE antibodies on the surface of mast cells and basophils. Understanding the cellular mechanisms that lead to high-affinity IgE production is required to develop better therapeutics for preventing this severe reaction. A recently discovered population of T follicular helper T13 cells regulates the production of high-affinity IgE in mouse models of allergy and can also be found in patients with allergies with IgE antibodies against food or aeroallergens.

IL-10-Dependent Crosstalk between Murine Marginal Zone B Cells, Macrophages, and CD8α Dendritic Cells Promotes Listeria monocytogenes Infection.

Type 1 CD8α conventional dendritic cells (cDC1s) are required for CD8 T cell priming but, paradoxically, promote splenic Listeria monocytogenes infection. Using mice with impaired cDC2 function, we ruled out a role for cDC2s in this process and instead discovered an interleukin-10 (IL-10)-dependent cellular crosstalk in the marginal zone (MZ) that promoted bacterial infection. Mice lacking the guanine nucleotide exchange factor DOCK8 or CD19 lost IL-10-producing MZ B cells and were resistant to Listeria.