Article
A mutation in the immunoproteasome subunit PSMB8 causes autoinflammation and lipodystrophy in humans.
The Journal of clinical investigation - 1 Oct 2011
Kitamura Akiko, Maekawa Yoichi, Uehara Hisanori, Izumi Keisuke, Kawachi Izumi, Nishizawa Masatoyo, Toyoshima Yasuko, Takahashi Hitoshi, Standley Daron M, Tanaka Keiji, Hamazaki Jun, Murata Shigeo, Obara Koji, Toyoshima Itaru, Yasutomo Koji
Abstract excerpt
Proteasomes are multisubunit proteases that play a critical role in maintaining cellular function through the selective degradation of ubiquitinated proteins. When 3 additional β subunits, expression of which is induced by IFN-γ, are substituted for their constitutively expressed counterparts, the structure is converted to an immunoproteasome. However, the underlying roles of immunoproteasomes in human diseases...
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