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Proteostasis disturbances within T cells from patients with PSMC3 variants were observed to correlate with an impairment in type I interferon (IFN) signaling in the same T cells; this impairment could be reversed by hindering the activity of the intracellular stress sensor protein kinase R (PKR). These findings suggest that a type I interferon response in patient-derived T cells was triggered by PKR activation, an effect of