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MRC Mitochondrial Biology Unit

 

Edmund Kunji

Professor of Biophysics

MRC Investigator

 

 

 

Mitochondrial transport proteins: Highly dynamic integral membrane proteins required for the translocation of metabolites and cofactors, key to the function of the mitochondrion

The outer membrane of mitochondria is permeable for small molecules as it contains large pores, but the inner membrane, which forms cristae, is tightly sealed. A large number of different molecules need to traverse the inner membrane to link the biochemical pathways of the cytosol and mitochondria. Among these compounds are keto acids derived from sugars and fatty acids derived from fat, which are oxidised inside mitochondria to generate the cellular fuel ATP. Amino acids, derived from protein, also enter mitochondria, where they are broken down, interconverted, or used in protein synthesis. Many vitamins are required in the mitochondrial matrix as they act as co-factors for many mitochondrial enzymes. Finally, nucleotides, which are required for replication and transcription of mitochondrial DNA, also need to traverse the inner membrane. More than 60 different transport proteins are present in the mitochondrial inner membrane to facilitate the translocation of these compounds. They belong to different protein families, such as the ABC transporter family, the mitochondrial pyruvate carrier family, and the mitochondrial carrier family. The function of the vast majority of them has not been established. We are interested in studying their role in cellular metabolism and human physiology. We would also like to establish how they work in order to identify the molecular cause for the diseases that are associated with their dysfunction.

Biography

Edmund Kunji obtained a Master’s degree in Biology (1990) and Chemistry (1991) and a Ph.D. in Mathematics and Natural Sciences (Cum Laude) from the University of Groningen, The Netherlands, carrying out research in the group of Bert Poolman. From 1996–2000 he was an EMBO Postdoctoral Fellow in the group of Richard Henderson at Medical Research Council, Laboratory of Molecular Biology, Cambridge. In 2000 he became a MRC Investigator at the MRC Mitochondrial Biology Unit, University of Cambridge, where he is still. In 2003 he received the Young Investigators Award of the EMBO and in 2005 he was elected Staff Fellow of Trinity Hall, University of Cambridge.

Publications

Selected Publications

Ruprecht JJ & Kunji ERS (2021)
 
Ruprecht JJ & Kunji ERS (2020)
TIBS, 45, 244-258

 

Kunji ERS, King MS, Ruprecht JJ, & Thangaratnarajah C. (2020)
Physiology, 35, 302-327. 

 

Ruprecht JJ, King MS, Zögg T, Aleksandrova AA, Pardon E, Crichton PG, Steyaert J & Kunji ERS. (2019)
Cell, 176(3), 435-447

 

 
Ruprecht JJ & Kunji ERS (2019)
Structural changes in the transport cycle of the mitochondrial ADP/ATP carrier.
Curr Opin Struct Biol 57, 135-144

 

Tavoulari S, Thangaratnarajah C, Mavridou V, Harbour ME, Martinou J-C & Kunji ERS (2019)
The yeast mitochondrial pyruvate carrier is a hetero-dimer in its functional state.
EMBO J 38, e100785

 

 

Publication profile - Google Scholar

PubMed (last 10 years)