Mycobacterial Disease and Impaired IFN-γ Immunity in Humans with Inherited ISG15 Deficiency

作者: Dusan Bogunovic , Minji Byun , Larissa A Durfee , Avinash Abhyankar , Ozden Sanal

DOI: 10.1126/SCIENCE.1224026

关键词: Interferon gammaAntibodyInterleukin 12ImmunologyInterferonSecretionImmunityIntracellularBiologyVirologyISG15

摘要: ISG15 is an interferon (IFN)-α/β-inducible, ubiquitin-like intracellular protein. Its conjugation to various proteins (ISGylation) contributes antiviral immunity in mice. Here, we describe human patients with inherited deficiency and mycobacterial, but not viral, diseases. The lack of production protein ISGylation was associated cellular susceptibility any viruses that tested, consistent the viral diseases these patients. By contrast, mycobacterium-induced secretion by leukocytes-granulocyte, particular-reduced IFN-γ lymphocytes, including natural killer cells, probably accounting for enhanced mycobacterial disease. This experiment nature shows largely redundant immunity, plays essential role as IFN-γ-inducing secreted molecule optimal antimycobacterial immunity.

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