Research Overview
Mitochondrial Function
Mitochondria are the powerhouses of most eukaryotic cells (nucleated cells) ----the membrane-contained structures (organelles) in that produce energy for cells----in the form of ATP. This is accomplished by oxidizing the major products of glycolysis, in which energy is extracted from sugars. Mitochondria contain their own DNA and resemble bacterial cells in their structure, and probably arose by means of early cells engulfing bacteria-like ancestors. In addition to energy production...
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Research Description
Research Specialties:
mitochondrial function, protein cycling
Mitochondria are intracellular organelles key to the physiology of eukaryotes. They provide energy to cells through the conversion of carbon sources into ATP, function in iron and calcium homeostasis, and play a central regulatory role in programmed cell death. Depending on the cell type and metabolic requirements of the cell, mitochondria exist in different shapes and numbers. The two most common shapes of mitochondria are long, filamentous networks and short, grain-like mitochondria (Figure 1). These forms occur in a dynamic equilibrium within a single cell. This equilibrium exists as mitochondria are continuously undergoing the opposing processes of fusion and fission, and the relative contribution of each process determines the overall degree of continuity of the network, as well as the average...
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Publications
Tondera D, Grandemange S, Jourdain A, Karbowski M, Mattenberger Y, Herzig S, Da CruzS, Clerc P, Raschke I, Merkwirth C, Ehses S, Krause F, Youle R, Chan DC, Alexander C, Langer T, Bauer C, Martinou JC. SLP-2 is required for stress-induced mitochondrial hyperfusion. EMBO J, 2009, in press.
Wang H, Karbowski M, and Monteiro MJ. Expanded polyglutamine proteins
induce mitochondrial fragmentation by disrupting mitochondrial fusion. Human
Molecular Genetics, 2009,
18:737-52
Zanna C, Ghelli, A, Porcelli AM, Karbowski M, Youle RJ,
Schimpf S, Wissinger B, Pinti M, Cossarizza A, Vidoni S, Valentino ML,
Rugolo M, Carelli V. OPA1 mutations associated with
dominant optic atrophy impair oxidative phosphorylation and
mitochondrial
fusion. Brain 2008, 131: 352-67
Karbowski M, Neutzner A, Youle
RJ. The mitochondria associated ubiquitin ligase MARCH5 is required for
Drp1-dependent division of mitochondria. J. Cell Biol. 2007, 178:
71-84 ("Mitochondria on the MARCH", an editorial on
the article was published in J. Cell Biol. 2007, 178:3)
Karbowski M, Norris K, Cleland
M, Jeong SY, Youle RJ. Role of Bax and Bak in mitochondrial morphogenesis. Nature 2006, 443: 658-662 ("Cell
biology: mitochondria shape up", an editorial on the article was published in Nature
2006, 443:646-7).
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