Janos Steffen

TL;DR: It is shown that i-proteasomes function to protect cell viability under conditions of IFN-induced oxidative stress, and it is found that the ubiquitylation machinery is concomitantly upregulated in response to IFNs, functioning to target defective ribosomal products (DRiPs) for degradation by i- Proteasome.

TL;DR: The identification of the transcription factor TCF11 (long isoform of Nrf1) as a key regulator for 26S proteasome formation in human cells to compensate for reduced proteolytic activity is reported.

TL;DR: It is shown that the main steps in 20S core complex formation take place at the endoplasmic reticulum (ER), and the proteasome maturation protein (POMP)—an essential factor of mammalian proteasomes biogenesis—interacts with ER membranes, binds to α1–7 rings, recruits β‐subunits stepwise and mediates the association of mammalian precursor complexes with the ER.

TL;DR: The genotype-phenotype correlation underscores the importance of SLC25A46 and fine tuning of mitochondrial fission and fusion in pontocerebellar hypoplasia and central neurodevelopment in addition to optic and peripheral neuropathy across the life span.

TL;DR: Mutation L341P causes rapid degradation of SLC25A46 by the ubiquitin-proteasome system, independent of activated stress pathways, including mitophagy and apoptosis.