Amorphic mutations in the recombination activating genes RAG1 and RAG2 have been
reported to cause T – B – SCID, whereas hypomorphic mutations led to the expansion of a few …

We previously reported the clinical phenotype of two siblings with a novel inherited developmental
and immunodeficiency syndrome consisting of severe intrauterine growth retardation …

The receptors for interferon-α/β (IFN-α/β) and IFN-γ activate components of the Janus kinase–signal
transducer and activator of transcription (JAK–STAT) signaling pathway, leading to …

Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the most common form of sporadic viral
encephalitis in western countries. Its pathogenesis remains unclear, as it affects otherwise …

Interleukin‐22 ( IL ‐22) has important functions in host defense at mucosal surfaces as well
as in tissue repair. It is unique as a cytokine that is produced by immune cells, including T‐…

Natural killer (NK) cells are circulating cytotoxic lymphocytes that exert potent and nonredundant
antiviral activity and antitumoral activity in the mouse; however, their function in host …

Five TLRs are thought to play an important role in antiviral immunity, sensing viral products
and inducing IFN-α/β and -λ. Surprisingly, patients with a defect of IRAK-4, a critical kinase …

Interleukin 22 (IL-22), which is produced by cells of the T H 17 subset of helper T cells and
other leukocytes, not only enhances proinflammatory innate defense mechanisms in epithelial …

A 2-year-old girl with recurrent severe varicella infections had a fatal outcome. Studies of
cellular and humoral immunity were normal. No natural killer (NK) cells were detected, and NK …

We describe four children with a novel primary immunodeficiency consisting of specific
natural-killer (NK) cell deficiency and susceptibility to viral diseases. One child developed an …