The homozygous founder Psmb8 variant of Nakajo-Nishimura syndrome/proteasome-associated autoinflammatory syndrome causes panniculitis-associated lipoatrophy and a shortened lifespan in mice.

A similar reduction was also observed in CD8+ T cells, although the difference did not reach statistical significance.

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MHC class I expression showed a trend toward reduction not only in immune cells, including B cells and T cells, but also in tissue-resident cells, which are included in CD11b + CD11c + and CD11b - CD11c + cell fractions, although these differences were not statistically significant (Fig. 6 A, B).

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