Hell Lab

Research

Vergrößern

We study several aspects of mitochondrial function and biogenesis, in particular the import of proteins into mitochondria and their sorting, folding and assembly within mitochondria. We focus on three major topics:

1.) The disulfide relay system in the intermembrane space of mitochondria

We want to understand how this systems transfers disulfide bonds into the substrate proteins. Moreover, we aim to identify additional substrates of the Mia40-Erv1 system and to analyse the effects of the disulfide relay system on the redox homeostasis of the intermembrane space.

2.) Structure und function of the TIM23 preprotein translocase and the mitochondrial import motor

The TIM23 preprotein translocase is the main translocase of the inner membrane. Our goal is to understand the mechanism and function of this translocase and its import motor. We employ biochemical, biophysical and genetic methods to elucidate how the components of the translocase interact and function to mediate translocation of preproteins.

3.) Structural and functional characterisation of the mitochondrial chaperone Hep1

We have identified a mitochondrial Hsp70 (mtHsp70) interacting protein, the Hsp70 escort protein (Hep1), which prevents the aggregation of mitochondrial Hsp70 chaperones. It is our aim to understand why mitochondrial Hsp70 chaperones have the propensity to aggregate and how this novel kind of chaperone protects them.

Publications

The Mia40 substrate Mix17 exposes its N-terminus to the cytosolic side of the mitochondrial outer membraneGenomic determinants of antigen expression hierarchy in African trypanosomes.
Resch M, Frickel JS, Dischinger K, Choo RSW, Hell K and Harner ME.
J Cell Sci. 2025 May 1;138(9):jcs263661.
PubMed

Dbi1 is an oxidoreductase and an assembly chaperone for mitochondrial inner membrane proteins.
Badrie S., Hell K. and Mokranjac D.
EMBO Rep. 2025 Feb;26(4):911-928.
PubMed

A single-cysteine mutant and chimeras of essential Leishmania Erv can complement the loss of Erv1 but not of Mia40 in yeast.
Specht S, Liedgens L, Duarte M, Stiegler A, Wirth U, Eberhardt M, Tomás A, Hell K, Deponte M.
Redox Biol. 2017; 15, 363-374.
PubMed

Methylation-controlled J-protein MCJ acts in the import of proteins into human mitochondria. Schusdziarra C., Blamowska M., Azem A, Hell K. Hum Mol Genet. 2013 Apr 1;22(7):1348-57.
PubMed

Divergent molecular evolution of the mitochondrial sulfhydryl:cytochrome C oxidoreductase Erv in opisthokonts and parasitic protists. Eckers E, Petrungaro C, Gross D, Riemer J, Hell K, Deponte M. J Biol Chem. 2013 Jan 25;288(4):2676-88.
PubMed

Biogenesis of the mitochondrial Hsp70 chaperone. Blamowska M, Neupert W, Hell K. J Cell Biol. 2012 Oct 1;199(1):125-35. doi: 10.1083/jcb.201205012. Epub 2012 Sep 24.
PubMed

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