Université de Fribourg

Proteasomal degradation induced by DPP9-mediated processing competes with mitochondrial protein import

Finger, Yannik ; Habich, Markus ; Gerlich, Sarah ; Urbanczyk, Sophia ; Logt, Erik ; Koch, Julian ; Schu, Laura ; Lapacz, Kim Jasmin ; Ali, Muna ; Petrungaro, Carmelina ; Salscheider, Silja Lucia ; Pichlo, Christian ; Baumann, Ulrich ; Mielenz, Dirk ; Dengjel, Jörn ; Brachvogel, Bent ; Hofmann, Kay ; Riemer, Jan

In: The EMBO Journal, 2020, vol. 39, no. 19, p. e103889

Plasticity of the proteome is critical to adapt to varying conditions. Control of mitochondrial protein import contributes to this plasticity. Here, we identified a pathway that regulates mitochondrial protein import by regulated N-terminal processing. We demonstrate that dipeptidyl peptidases 8/9 (DPP8/9) mediate the N-terminal processing of adenylate kinase 2 (AK2) en route to mitochondria....

Université de Fribourg

The re-emerging pathophysiological role of the cystathionine-β-synthase - hydrogen sulfide system in Down syndrome

Szabo, Csaba

In: The FEBS Journal, 2020, p. -

Down syndrome (DS) is associated with significant perturbances in many morphological and biochemical features. Cystathionine‐β‐synthase (CBS) is one of the key mammalian enzymes that is responsible for the biological production of the gaseous transmitter hydrogen sulfide (H2S). When H2S is overproduced, it can exert detrimental cellular effects, in part due to inhibition of mitochondrial...