Visual Universitätsmedizin Mainz

Konstanze Winklhofer, MD, PhD


Professor and Chair of Molecular Cell Biology
Ruhr University Bochum, Germany 


The Winklhofer lab is interested in cellular quality control and stress response pathways implicated in the maintenance of neuronal viability and homeostasis. By studying the function and dysfunction of genes associated with neurodegenerative diseases the lab aims at identifying key neuroprotective pathways that can be targeted for disease-modifying strategies. From these studies it emerged that several Parkinson's disease-associated genes converge to maintain mitochondrial integrity by modulating mitochondrial stress response and quality control pathways. Another research focus are the causes and consequences of protein misfolding and the regulation of the proteostasis network. In this context, the role of linkage-specific ubiquitination at the interface between protein quality control and innate immune signaling is investigated.

The Winklhofer Lab

Selected workshop-related publications

A protein quality control pathway regulated by linear ubiquitination.
EMBO Journal.38(9): e100730. doi:10.15252/embj.2018100730
van Well EM, Bader V, Patra M, Sánchez-Vicente A, Meschede J, Furthmann N, Schnack C, Blusch A, Longworth J, Petrasch-Parwez E, Mori K, Arzberger T, Trümbach D, Angersbach L, Showkat C, Sehr DA, Berlemann LA, Goldmann P, Clement AM, Behl C, Woerner AC, Saft C, Wurst W, Haass C, Ellrichmann G, Gold R, Dittmar G, Hipp MS, Hartl FU, Tatzelt J, Winklhofer KF. (2019)

PERK activation mitigates tau pathology in vitro and in vivo.
EMBO Molecular Medicine
. 9(3): 371-384 doi: 10.15252/emmm.201606664
Bruch J, Xu H, Rösler TW, De Andrade A, Kuhn PH, Lichtenthaler SF, Arzberger T, Winklhofer KF, Müller U, Höglinger GU (2017)

Cytoplasmic protein aggregates interfere with nucleo-cytoplasmic transport of protein and RNA.
Science 351(6269): 173-176 doi: 10.1126/science.aad2033. 
Woerner AC, Frottin F, Hornburg D, Feng LR, Meissner F, Patra M, Tatzelt J, Mann M, Winklhofer KF, Hartl FU and Hipp MS (2016)

Parkin cooperates with GDNF/RET signaling to prevent dopaminergic neuron degeneration
Journal of Clinical Investigation 125(5):1873-85. doi: 10.1172/JCI79300
Meka DP, Müller-Rischart AK, Nidadavolu P, Mohammadi B, Motori E, Ponna SK, Aboutalebi H, Bassal M, Annamneedi A, Finckh B, Miesbauer M, Rotermund N, Lohr C, Tatzelt J, Winklhofer KF and Kramer ER (2015)

The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO
Molecular Cell 7;49(5):908-21. doi: 10.1016/j.molcel.2013.01.036
Müller-Rischart AK, Pilsl A, Beaudette P, Hadian K, Deinlein A, Funke M, Patra M, Motori E, Schweimer C, Kuhn PH, Hrelia S, Lichtenthaler SF, Wurst W, Trümbach D, Langer T, Krappmann D, Dittmar G, Tatzelt, J and Winklhofer KF (2013)



Selected Reviews

Parkin and mitochondrial quality control: Towards assembling the puzzle.
Trends in Cell Biology 24(6):332-41. doi: 10.1016/j.tcb.2014.01.001.
Winklhofer KF (2014)

Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences.
Embo Journal 26; 31(14):3038-62. doi: 10.1038/emboj.2012.170.
Exner N, Lutz AK, Haass C, Winklhofer KF. (2012)