Kunz Workgroup - Clinical Neurochemistry

Our research group is engaged in deciphering problems of mitochondrial energy metabolism as a potential cause of human CNS diseases, identifying potential mitochondria-targeted neuroprotective strategies and creating a diagnostic platform for metabolism-related forms of genetic epilepsy.

The following research topics are the focus of the laboratory:

•         Mitochondrial dysfunction in relation to epilepsy, neurodegenerative diseases, mitochondrial diseases and ageing.
•         Genetics of mitochondrial DNA and the origin of mutations in mitochondrial DNA
•         Brain energy metabolism as a potential target of neuroprotective strategies
•         Identification of genomic variants associated with epilepsy

Staff:

• Dr. Gábor Zsurka
• Dr. Alexei Kudin
• Dipl.-Biol. Kerstin Hallmann
• Susanne Beyer
• Silke Schiller
• Genevieve Trombly (Doktorandin)
• Afaf Milad (Doktorandin)
• Nika Atanelov (Masterstudent)

Current publications (selected):

• Rotko D, Kudin AP, Zsurka G, Kulawiak B, Szewczyk A, Kunz WS. Molecular and Functional Effects of Loss of Cytochrome c Oxidase Subunit 8A. Biochemistry (Moscow), 2021, Vol. 86, No. 1, pp. 3343. DOI: 10.1134/S0006297921010041.
• von Wrede R, Jeub M, Ariöz I, Elger CE, von Voss H, Klein HG, Becker AJ, Schoch S, Surges R, Kunz WS. Novel KCNH1 Mutations Associated with Epilepsy: Broadening the Phenotypic Spectrum of KCNH1-Associated Diseases. Genes (Basel). 2021 Jan 21;12(2):132. doi: 10.3390/genes12020132.
• Szibor M, Gizatullina Z, Gainutdinov T, Endres T, Debska-Vielhaber G, Kunz M, Karavasili N, Hallmann K, Schreiber F, Bamberger A, Schwarzer M, Doenst T, Heinze HJ, Lessmann V, Vielhaber S, Kunz WS, Gellerich FN. Cytosolic, but not matrix, calcium is essential for adjustment of mitochondrial pyruvate supply. J Biol Chem. 2020 Apr 3;295(14):4383-4397. doi: 10.1074/jbc.RA119.011902.
• Schlapakow E, Peeva V, Zsurka G, Jeub M, Wabbels B, Kornblum C, Kunz WS. Distinct segregation of the pathogenic m.5667G>A mitochondrial tRNA Asn mutation in extraocular and skeletal muscle in chronic progressive external ophthalmoplegia. Neuromuscul Disord. 2019 May;29(5):358-367. doi: 10.1016/j.nmd.2019.02.009.
• Peeva V, Blei D, Trombly G, Corsi S, Szukszto MJ, Rebelo-Guiomar P, Gammage PA, Kudin AP, Becker C, Altmüller J, Minczuk M, Zsurka G, Kunz WS. Linear mitochondrial DNA is rapidly degraded by components of the replication machinery. Nat Commun. 2018 Apr 30;9(1):1727. doi: 10.1038/s41467-018-04131-w.

PubMed-listed publications of the neurochemistry lab: here

Grants:

• Mechanisms of mitochondrial DNA deletion - The role of linear mtDNA (DFG, 2014 -)
• Non-canonical mtDNA species in mitochondrial dysfunction and oxidative damage (DFG, 2019 - )