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Associate Professor (with tenure)Associate Professor of Neurology
Primary Office: 2100 RCPIowa City, IA 52242
Email: email@example.comWeb: Lab Website
MD, University of Malaga Medical School, Malaga, Spain
Fellowship, Movement Disorders, University of Iowa Hospitals and Clinics, Iowa City, IAInternship, St. Vincent Hospital, Worcester, MAResidency, Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA
Medical License, Iowa Board of Medical Examiners Iowa Controlled Substance, DEAECFMG Certification, Educational Commission for Foreign Medical Graduates
Biosciences Graduate ProgramInterdisciplinary Graduate Program in GeneticsInterdisciplinary Graduate Program in Molecular and Cellular BiologyInterdisciplinary Graduate Program in NeuroscienceMedical Scientist Training Program
Dr. Gonzalez-Alegre's research aims to understand the biological basis of neurological disorders caused by dysfunction of the basal ganglia, and to develop novel therapies for this group of neurological diseases. Currently, studies focus on a disease known as DYT1 dystonia, the most common form of inherited dystonia. DYT1 dystonia, a dominantly inherited, incurable disease, is caused by a three-nucleotide deletion in the gene TOR1A that causes the loss of a glutamic acid in the protein torsinA, a AAA protein (ATPases Associated with diverse cellular Activities) that resides primarily in the endoplasmic reticulum. However, torsinA carrying the disease-causing mutation accumulates in the nuclear envelope. Analyses of dominant negative mutants suggest that torsinA normally functions within the perinuclear space of the nuclear envelope. In addition to torsinA, the mammalian torsin family of proteins includes torsinB, torsin-related protein 2A (torp2A) and torp3A. Over the next few years, my research will focus on DYT1 dystonia through three major projects: 1) Defining the biological role of torsinA in the nuclear envelope; 2) Characterize the expression and functional properties of the torsin gene family; 3) Development of therapeutic RNAi interference for DYT1 dystonia. Understanding the pathobiology underlying DYT1 dystonia could help us understand the biological basis for other forms of dystonia and the growing list of nuclear envelope-related human diseases, as well as reveal fundamental biological issues in nuclear envelope function and functional organization of the basal ganglia. In addition, these studies will contribute to exploit RNA interference as a therapy for DYT1 dystonia and other incurable neurological diseases.
Center for Gene Therapy of Cystic Fibrosis and other Genetic Diseases
The ubiquitin ligase F-box/G-domain protein 1 promotes the degradation of the disease-linked protein torsinA through the ubiquitin-proteasome pathway and macroautophagy.
2012 November. 224:160-71.
Miniature release events of glutamate from hippocampal neurons are influenced by the dystonia-associated protein torsinA..
Synapse (New York, N.Y.).
2012 September. 66(9):807-22.
Substance abuse may be a risk factor for earlier onset of Huntington disease..
Journal of neurology.
2012 September. 259(9):1824-31.
Clinical predictors of driving status in Huntington's disease.
Movement disorders : official journal of the Movement Disorder Society.
2012 August. 27(9):1146-52.
Synaptic vesicle recycling is enhanced by torsinA that harbors the DYT1 dystonia mutation..
Synapse (New York, N.Y.).
2012 May. 66(5):453-64.
DYT1 knock-in mice are not sensitized against mitochondrial complex-II inhibition..
Secondary dystonia in a botulinum toxin clinic: clinical characteristics, neuroanatomical substrate and comparison with idiopathic dystonia..
Parkinsonism & related disorders.
2011 December. 17(10):749-52.
A functional role for TorsinA in herpes simplex virus 1 nuclear egress..
Journal of virology.
2011 October. 85(19):9667-79.
Lethal toxicity caused by expression of shRNA in the mouse striatum: implications for therapeutic design..
2011 July. 18(7):666-73.
Shooting the Messenger (RNA) in the Neurology Clinic: The Promise of Therapeutic RNA Interference..
AAN.com (webpage of the American Academy of Neurology), Science Editorials and Reviews.
2008 January 4.
Date Last Modified: 08/27/2013 -
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