Molecular pathogenesis of tumorigenesis caused by succinate dehydrogenase defect |
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| Affiliation: | 1. Department of Biology, Maynooth University, National University of Ireland, Maynooth W23F2H6, Co. Kildare, Ireland;2. Kathleen Lonsdale Institute for Human Health Research, Maynooth University, Maynooth W23F2H6, Co. Kildare, Ireland;3. Institute of Physiology II, University of Bonn, D53115 Bonn, Germany;4. National Institute for Cellular Biotechnology, Dublin City University, Dublin 9, Ireland;1. Laboratório de Biologia Celular, Instituto Oswaldo Cruz, Fiocruz, Rio de Janeiro, RJ, Brazil;2. Laboratório de Inovações em Terapias, Ensino e Bioprodutos, Instituto Oswaldo Cruz, Fiocruz, Rio de Janeiro, RJ, Brazil;3. Laboratório de Imunoreceptores e Sinalização, Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil;4. Laboratório de Biologia Molecular e Doenças Endêmicas, Instituto Oswaldo Cruz, Fiocruz, Rio de Janeiro, RJ, Brazil;5. Laboratório de Imunofarmacologia, Instituto Oswaldo Cruz, Fiocruz, Rio de Janeiro, RJ, Brazil;6. Laboratório de Biologia Celular, Departamento de Biologia, Universidade Federal de Juiz de Fora, Juiz de Fora, MG, Brazil;1. Cell Physiology Laboratory, Institute of Biomedical Problems, Russian Academy of Sciences, Khoroshevskoye Shosse, 76a, 123007 Moscow, Russia;2. Faculty of Basic Medicine, Moscow State University, Lomonosovsky Prospekt, 31-5, 117192, Moscow, Russia;1. Department of Biology, Colgate University, Hamilton, NY, 13346, USA;2. Section on Molecular Transport, National Institute of Childhood Health and Human Development, Bethesda, MD, 20814, USA;3. Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, 80045, USA;4. Department of Molecular and Computational Biology, University of Southern California, Los Angeles, CA 90007, USA;5. Harvard Medical School, 200 Longwood Ave, Boston, MA 02115, USA;6. SUNY Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, NY 11203, USA;7. School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA;1. Key Laboratory of Pesticide & Chemical Biology of Ministry of Education, College of Chemistry, Central China Normal University, Wuhan, 430079, PR China;2. Laboratory of Organometallics, Catalysis and Ordered Materials, State Key Laboratory of Advanced Technology for Materials Synthesis and Processing, Wuhan University of Technology, Wuhan, 430070, PR China |
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| Abstract: | Succinate dehydrogenase (SDH), also named as complex II or succinate:quinone oxidoreductases (SQR) is a critical enzyme in bioenergetics and metabolism. This is because the enzyme is located at the intersection of oxidative phosphorylation and tricarboxylic acid cycle (TCA); the two major pathways involved in generating energy within cells. SDH is composed of 4 subunits and is assembled through a multi-step process with the aid of assembly factors. Not surprisingly malfunction of this enzyme has marked repercussions in metabolism leading to devastating tumors such as paraganglioma and pheochromocytoma. It is already known that mutations in the genes encoding subunits lead to tumorigenesis, but recent discoveries have indicated that mutations in the genes encoding the assembly factors also contribute to tumorigenesis. The mechanisms of pathogenesis of tumorigenesis have not been fully understood. However, a multitude of signaling pathways including succinate signaling was determined. We, here discuss how defective SDH may lead to tumor development at the molecular level and describe how yeast, as a model system, has contributed to understanding the molecular pathogenesis of tumorigenesis resulting from defective SDH. |
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| Keywords: | Succinate dehydrogenase Mitochondria Signaling ROS Hereditary paraganglioma Pheochromocytoma |
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