BBA - Molecular Basis of Disease - Mitochondrial Disease
BBA - Molecular Basis of Disease Mitochondrial Disease Volume 1792, Issue 12, Pages 1095-1212 (December 2009)
Edited by H.T. Jacobs and J.A.M. Smeitink
In this special section of BBA—Molecular Basis of Disease a distinguished panel of expert scientists present their current outlook on mitochondrial disease, covering the various topics outlined above. Whilst progress is always slower than one might hope, we believe that the application of modern techniques in molecular biology will resolve most of the outstanding questions about mitochondrial disease in the coming decades. In addition, the growing co-operation in the field between clinical and basic scientists and between experts in many countries opens the prospect of a rapid translation of discovery into practical health benefits
Howy Jacobs
Howy Jacobs is Academy Professor at the Institute of Medical Technology, University of Tampere, Finland. After obtaining his PhD in Glasgow, Scotland, in 1981, he conducted postdoctoral research in the California Institute of Technology, returning to Glasgow as a Royal Society University Research Fellow for a decade before taking up his appointment in Finland in the mid 1990s. His research is focused on studying the molecular pathophysiology of mitochondrial disease, using both mammalian cell and fly models. A recent development in this work is the application of respiratory chain by-pass enzymes from lower eukaryotes to alleviate inhibition of the mitochondrial oxidative phosphorylation system. In addition, Howy maintains a long-standing interest in the mechanics of mitochondrial DNA replication. These various projects are conducted under the aegis of the Academy of Finland Centre of Excellence in Research on Mitochondria, Disease and Ageing, comprising four research groups based in Tampere and in Helsinki, which Howy leads. Howy is also an EMBO member. He currently serves as senior editor of EMBO reports, EMBO's prestige journal focusing on ground-breaking, short-format research papers, in-depth scientific reviews and commentary on issues at the interface of science and society.
Jan Smeitink
Jan Smeitink, MD, PhD, director of the Nijmegen Centre for Mitochondrial Disorders (www.ncmd.nl) and the Institute for Genetic and Metabolic Disease (www.igmd.nl) of the Radboud University Nijmegen Medical Centre, The Netherlands. Prof. Smeitink received his PhD in 1992 at the Radboud University. As a pediatrician he specialized in inborn errors of metabolism with special emphasis on mitochondrial medicine. He is a foreign adjunct professor at the Karolinska Insitute, Stockholm, Sweden. His research focuses on oxidative phosphorylation disorders in particular on complex I. As such he contributed to the characterization of structural and assembly genes and the cell biological consequences of human complex I disease. His final goal is to make a substantial contribution to the development of new treatment modalities for oxidative phosphorylation disorders. Recently he initiated the centre for systems biology and bioenergetics (www.csb-bioenergetics.nl).
