The Molecular Aspects of the Mitochondrial Chaperone HSP60 Dysfunction

2021.11.11 | Graduate School of Health

On Thursday 25 November at 15:15, Cagla Cömert defends her dissertation entitled "The Molecular Aspects of the Mitochondrial Chaperone HSP60 Dysfunction".

Besides providing the majority of ATP production in cells, mitochondria are involved in many other cellular functions and central for cellular stress signaling. As mitochondrial molecular chaperones, the HSP60/HSP10 complex interacts with mitochondrial matrix proteins to facilitate their folding and maintenance; thus, the impairment of the HSP60/HSP10 complex causes mitochondrial dysfunctions due to the extreme dependence of some mitochondrial proteins for their folding on the complex. Furthermore, HSP60 deficiency is observed as rare monogenic diseases in patients. These rare diseases show that different variations and types of inheritance are associated with different phenotypes and disease severity; however, hypomyelination, i.e defective isolation of nerve fibers, are the commonly observed phenotype.

In this PhD project, Cagla Cömert has investigated the molecular mechanisms triggered by HSP60 deficiency and their contribution to disease in I) a model cell line, II) a CRISPR/Cas9 generated hspd1 knockout zebrafish animal model, III) a patient-derived fibroblast cell line generated from a patient carrying a hypomyelination-associated HSP60 variation, combining transcriptomics, proteomics, metabolomics, and functional analyses.

The summary is written by the PhD student.     

The defence is public and takes place in Auditorium J116-113, Entrance J, Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, Aarhus N and online. Please read full press release for more information. 

Contact:

PhD student Cagla Cömert

Mail: cagla.comert@clin.au.dk

Phone: (+45) 81930737

Read full press release