AU Studieprojekt: Mitochondrial Dysfunction in Diseases

Aarhus Universitet (AU)

Studieprojekt/speciale
Østjylland (Aarhus)

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Mitochondrial Dysfunction in Diseases Peter Bross Research Unit for Molecular Medicine peter.bross@clin.au.dk Publications Background Our lab focuses on mitochondrial function and mechanisms of mitochondrial dysfunction in diseases. Besides providing the majority of ATP production in cells, mitochondria are also involved in many other cellular functions and central for cellular stress signaling. Mitochondrial dysfunction induces not only inherited mitochondrial disorders but also contributes to neurodegenerative diseases, cancer, diabetes, and metabolic syndrome. Cell models used in the projects Mitochondrial functions and proteins Questions • • What are the molecular mechanisms of mitochondrial dysfunctions caused by deficiencies of mitochondrial proteins? Does mitochondrial profile differ between patient and control cells? Techniques • • • • Using control and patient peripheral blood mononuclear cells (PBMCs) to profile mitochondrial respiration. Characterization of MCAD (Medium-chain acyl-CoA dehydrogenase) deficiency in IPS cells. Mitochondrial phenotyping of cultured patient fibroblasts with molecular biology techniques. Analyzing the mitochondrial deficiency in mutant zebrafish embryos by proteomics. 1 00 95 90 85 80 75 70 65 60 55 50 45 40 35 30 25 20 15 10 5 0 3 00 350 400 450 500 550 600 650 700 750 m/z Mitochondrial bioenergetics Proteomics & Western Blot Image Cytometry Genotyping Possible Bachelor & Master Project topics • • • • Analysis of mitochondrial and cellular bionergetics in PBMCs as marker for health status. Studies of the liver pathology of a fatty acid oxidation disorder using IPS cell derived MCAD mutant hepatocytes. Molecular phenotypes in fibroblast cells from patients with deficiency of the mitochondrial HSP60 chaperone. Investigating the proteins affected by the deficiency of HSP60 chaperone by proteomics in a zebrafish animal model. References 1. Bross, P., & Fernandez-Guerra, P. (2016). Disease-Associated Mutations in the HSPD1 Gene Encoding the Large Subunit of the Mitochondrial HSP60/HSP10 Chaperonin Complex PubMed 2 3 . . Bross, P., & Palmfeldt, J. (2017). Proteomics of human mitochondria PubMed Fernandez-Guerra P. et al. (2015). Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts from Patients with Inherited Disorders. PubMed 4 . . Cömert, C., Fernandez-Guerra, P. & Bross, P. (2018). A Cell Model for HSP60 Deficiencies: Modeling Different Levels of Chaperonopathies Leading to Oxidative Stress and Mitochondrial Dysfunction. PubMed Y. Zhou, Y. et al. (2017). Mitochondrial Spare Respiratory Capacity Is Negatively Correlated with Nuclear Reprogramming Efficiency, Stem cells and development, 26 166-176. PubMed 5


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Studieprojekt/speciale
Østjylland (Aarhus)
Fødevarer & Veterinær
Kemi, Biotek & Materialer
Medicinal & Sundhed
Forskning & Udvikling
Medicinal & Sundhed
Naturvidenskab



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Aarhus Universitet (AU)
Aarhus Universitet (AU)
Aarhus Universitet blev grundlagt i 1928 og er i dag i den absolutte verdenselite på flere forskningsfelter. Aarhus Universitet (AU) er blandt verdens 10 bedste universiteter grundlagt inden for de seneste 100 år og har en lang tradition for partnerskaber med nogle af verdens bedste forskningsinstitutioner og universitetsnetværk.

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