Juan Pérez RodrÃguez
Professor
Department of Cell Biology, Genetics and Physiology
Universidad de Malaga
Spain
Biography
Juan Pérez Rodríguez (j Perez), is a graduate and doctor (1985) in Biological Sciences by the University of Malaga. Trainee Researcher Training in the Department of Cell Biology. Full Professor of Physiology in 1989. Professor of Physiology since 2010. His scientific career has been completed with several research stays at the University of Cambridge, UK, between 2005 and today. His current research focuses on the study of the molecular basis of serpinopathies: conformational diseases produced by structural alterations in serpins (serine protease inhibitors). Certain point mutations give rise to poorly folded molecules that have little or no functionality and can cause serious pathological alterations. For ex., The so-called alpha-1-antitrypsin Z variant (AAT, protease inhibitor elastase), a mutation that affects a single amino acid, causes polymerization of the protein in the endoplasmic reticulum of the hepatic producing cells which can trigger hepatic cirrhosis . In addition, individuals carrying the Z mutation are likely to contract pulmonary emphysema due to poor control of elastase activity.
Research Interest
Generation of monoclonal antibodies (mAbs) against normal and mutant snakes.
Publications
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Miranda E, McLeod I, Davies MJ, Perez J , Römisch K, Crowther DC, Lomas DA. The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. Human Molecular Genetics 1: 17 (11): 1527-39. (2008).
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Kroeger H, Miranda E, MacLeod I, Perez J , Crowther DC, Marciniak SJ, Lomas DA. Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins. Journal of Biological Chemistry 284 (34): 22793-802. (2009).
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Ekeowa UI, Freeke J, Miranda E, Gooptu B, Bush MF, Perez J , Teckman J, Robinson CV, Lomas DA. Defining the mechanism of polymerization in the serpinopathies. Proc Natl Acad Sci USA 107 (40): 17146-51 (2010).
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Miranda E, Perez J , Ekeowa UI, Hadzic N, Kalsheker N, Gooptu B, Portmann B, Belorgey D, Hill M, Chambers S, Teckman J, Alexander GJ, Marciniak SJ, Lomas DA. A Novel Monoclonal Antibody to Characterize Pathogenic Polymers in Liver Disease Associated with alpha 1-Antitrypsin Deficiency. Hepatology 52 (3): 1078-88 (2010).
