[HTML][HTML] Immunoproteasomes preserve protein homeostasis upon interferon-induced oxidative stress

…, A Voigt, F Schröter, T Prozorovski, N Lange, J Steffen… - Cell, 2010 - cell.com
Interferon (IFN)-induced immunoproteasomes (i-proteasomes) have been associated with
improved processing of major histocompatibility complex (MHC) class I antigens. Here, we …

[PDF][PDF] Proteasomal degradation is transcriptionally controlled by TCF11 via an ERAD-dependent feedback loop

J Steffen, M Seeger, A Koch, E Krüger - Molecular cell, 2010 - cell.com
Coordinated regulation of the ubiquitin-proteasome system (UPS) is crucial for the cell to
adjust its protein degradation capacity to changing proteolytic requirements. We have shown …

The proteasome maturation protein POMP facilitates major steps of 20S proteasome formation at the endoplasmic reticulum

B Fricke, S Heink, J Steffen, PM Kloetzel, E Krüger - EMBO reports, 2007 - embopress.org
The quality control of proteins mediated by the plasticity of the proteasome system is
regulated by the timely and flexible formation of this multisubunit proteolytic enzyme complex. …

Loss of function of SLC25A46 causes lethal congenital pontocerebellar hypoplasia

J Wan, J Steffen, M Yourshaw, H Mamsa, E Andersen… - Brain, 2016 - academic.oup.com
Disturbed mitochondrial fusion and fission have been linked to various neurodegenerative
disorders. In siblings from two unrelated families who died soon after birth with a profound …

Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria

J Steffen, AA Vashisht, J Wan, JC Jen… - Molecular biology of …, 2017 - Am Soc Cell Biol
SCL25A46 is a mitochondrial carrier protein that surprisingly localizes to the outer membrane
and is distantly related to Ugo1. Here we show that a subset of SLC25A46 interacts with …

Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1

N Miyata, J Steffen, ME Johnson… - Proceedings of the …, 2014 - National Acad Sciences
Primary hyperoxaluria 1 (PH1; Online Mendelian Inheritance in Man no. 259900), a typically
lethal biochemical disorder, may be caused by the AGT P11LG170R allele in which the …

[HTML][HTML] The mitochondrial fission protein Drp1 in liver is required to mitigate NASH and prevents the activation of the mitochondrial ISR

J Steffen, J Ngo, SP Wang, K Williams, HF Kramer… - Molecular …, 2022 - Elsevier
Objective The mitochondrial fission protein Drp1 was proposed to promote NAFLD, as inhibition
of hepatocyte Drp1 early in life prevents liver steatosis induced by high-fat diet in mice. …

[HTML][HTML] ER–mitochondria contacts: Actin dynamics at the ER control mitochondrial fission via calcium release

J Steffen, CM Koehler - The Journal of cell biology, 2018 - ncbi.nlm.nih.gov
The formin-like protein INF2 is an important player in the polymerization of actin filaments. In
this issue, Chakrabarti et al.(2018. J. Cell Biol. https://doi. org/10.1083/jcb. 201709111) …

Stendomycin selectively inhibits TIM23-dependent mitochondrial protein import

I Filipuzzi, J Steffen, M Germain, L Goepfert… - Nature chemical …, 2017 - nature.com
Tim17 and Tim23 are the main subunits of the TIM23 complex, one of the two major essential
mitochondrial inner-membrane protein translocon machineries (TIMs). No chemical probes …

TCF11 at the crossroads of oxidative stress and the ubiquitin proteasome system

A Koch, J Steffen, E Krüger - Cell Cycle, 2011 - Taylor & Francis
It is well established that mutual relations exist between the oxidative status of a cell and the
ubiquitin proteasome system (UPS). Oxidation of proteins leads to misfolding and thus …