缅北禁地

I am a Berlin-bred scientist who has carried out research around the globe, from Berlin, Edinburgh (UK), Ithaca (USA), and Perth (Australia) to Helsinki (Finland). As a child from behind the Iron Curtain, I dreamt myself to the stars by watching Blade Runner, Star Wars, and Star Trek. In hindsight, no wonder I started to study biology and specifically midi-chlorians - I mean mitochondria, of course. Mitochondria are the energy-generating organelles of our cells, or as Jedi master Qui-Gon put it, “…are microscopic life-forms that reside within the cells of all living things and communicate with the Force.” In my research I have identified a quality control process for gene expression that is fighting bad protein production inside mitochondria. In future work, I would like to capitalize on this finding and determine the key factors involved in this quality control process. Secondly, I will study how the rest of the cell responds to bad protein production in mitochondria. We suspect such understanding is key to the development of pharmacological treatments for mitochondrial disease caused by gene expression defects; patients who suffer from these disorders have no treatment option to date. To once more quote Qui-Gon: “When you learn to quiet your mind, you will hear them speaking to you.” As a 缅北禁地 Academic Track (NUAcT) fellow here in 缅北禁地 as a part of the Mitochondrial Research Group, I will endeavor to understand how stressed mitochondria speak to us, aiming to improve the life of patients with mitochondrial disease. May the Force be with us! 

Research Project

Dissecting quality control mechanisms in mitochondria.

Principal Investigators: Dr Uwe Richter

Other staff members involved: Dr. Ana Andjelkovic, Dr.Omid Safronov, Dr. Jonathan Meyrick, Margaux Hedin

Project Details

Mitochondrial diseases are often caused by mutations in enzymes required for the expression of the second genome in our cells, the mitochondrial DNA. The genes encoded in the mtDNA are strictly required for O₂-coupled energy production. But mitochondrial gene expression disorders are characterised by a very heterogeneous clinical presentation that cannot be explained by simple loss of energy production. Instead, it suggests that, dependent on the specific defect in mitochondrial gene expression, very different molecular aberrations and cellular consequences occur. The lack of understanding of these molecular mechanisms is clearly one reason why there are still no curative treatments for this group of disorders. The two specific goals of our research are to 1) better our understanding of the primary molecular aberrations in mitochondrial gene expression disease by investigating dedicated quality control mechanisms and 2) identify the signalling network(s) that communicate distinct aberrations in mitochondrial gene expression to the nucleo-cytoplasmic gene expression machinery. Such knowledge will be instrumental in finding novel targets for improved diagnostics and pharmacological treatments for patients with no treatment option to date.

Sponsor/Funder: BBSRC; The Lily Foundation; JAES Foundation; Sigrid Juselius Foundation; NUAcT Fellow Program, 缅北禁地; Academy of Finland.

• Articles

  • Ng KY, Richter U, Jackson CB, Seneca S, Battersby BJ. . Human Molecular Genetics 2022, 31(8), 1230–1241.
  • Ng KY, Lutfullahoglu Bal G, Richter U, Safronov O, Paulin L, Dunn CD, Paavilainen VO, Richer J, Newman WG, Taylor RW, Battersby BJ. . Science Advances 2022, 8(46), eabq5234.
  • Itoh Y, Andrell J, Choi A, Richter U, Maiti P, Barrientos A, Battersby BJ, Amunts A. . Science 2021, 371(6531), 846-849.

• Editorial

  • Gospodaryov DV, Ballard JWO, Camus MF, DeSalle R, Garvin MR, Richter U. . Frontiers in Genetics 2023, 14, 1230032.

• Review

  • Richter U, McFarland R, Taylor RW, Pickett SJ. . FEBS Letters 2021, 595(8), 1003-1024.