Raymond G. Perelman Center for
Cellular and Molecular Therapeutics

Gonzalez-Alegre Laboratory

Pedro Gonzalez-Alegre

Pedro Gonzalez-Alegre MD, PhD

Dr. Gonzalez-Alegre is a faculty member of the Center for Cell and Molecular Therapy at the Children’s Hospital of Philadelphia and Associate Professor of Neurology at the University of Pennsylvania. Dr. Gonzalez-Alegre is a physician-scientist with clinical expertise on movement disorders and neurogenetics. He directs the Huntington’s Disease and Neurogenetics clinics at the University of Pennsylvania. Dr. Gonzalez-Alegre is a member of the American Academy of Neurology (AAN) and the Movement Disorders Society (MDS), and a Fellow of the American Neurological Association (ANA). Dr. Gonzalez-Alegre has received the S. Weir Mitchell Award of the AAN, Junior Award for Excellence in Basic Science Research from the MDS, the Jon Stolk Award of the AAN and the Stanley Fahn Award of the Dystonia Medical Research Foundation.

The long-range goal of research in the Gonzalez-Alegre Laboratory is to develop treatments for human diseases caused by basal ganglia dysfunction based on biological information gained from studies performed in cellular and animal systems and in patients seen in the clinic. We focus on the study of inherited diseases, mostly dystonia, Huntington’s Disease and inherited ataxias.

Our research on inherited dystonia uses cellular and animal models to better understand the neurobiological process underlying this movement disorder. By investigating different genetic forms of this disorder, such as DYT1 and DYT6, we aim to identify shared pathogenic pathways. This might help us devise therapeutic strategies that could be applicable to different forms of dystonia. In addition, we are developing gene silencing strategies, such as RNAi and antisense oligonucleotides, as potential therapies for specific genetic forms of dystonia.

Through collaborative translational work with other member of the Center for Cell and Molecular Therapy, Dr. Gonzalez-Alegre directs the efforts to design and perform early phase clinical trials in patients with neurological disease using molecular therapeutic approaches such as gene replacement or silencing.

Positions

Year Position
1996 MD, PhD (Neuroscience, 2009): University of Malaga (Spain)
1998-1999 Intern, Department of Medicine. Saint Vincent Hospital. Worcester, MA
1999-2002 Resident, Department of Neurology, University of Iowa Hospitals and Clinics. Iowa City, IA
2002-2004 Postdoctoral Fellow, Department of Neurology. University of Iowa Hospitals and Clinics
2004-2013 Assistant Professor, Department of Neurology. University of Iowa
2011-2014 Member, Executive Committee, Graduate Program in Neuroscience. University of Iowa
2012-2014 Director, Residency Program, Department of Neurology. University of Iowa
2013-2014 Associate Professor with tenure, Department of Neurology. University of Iowa
2014-present Associate Professor, Department of Neurology. University of Pennsylvania
2014-present Associate Professor, Center for Cellular & Molecular Therapeutics, The Children Hospital of Philadelphia

Pedro Gonzalez-Alegre, MD, PhD

Post-doctoral Research Fellow

Genevieve Beauvais, PhD

Lab Support

Jaime Watson, MS: Lab Manager

Graduate Students

Name Current Position
Janine Martin 2006-2011, Genetics
Kara Gordon 2007-2011, Neuroscience
Ginny Harris 2007-2010, MSTP, Molecular & Cellular Biology

Rotation Graduate Students

Name Current Position
Pamela Wernett (2006) Neuroscience Graduate Program
Marlene Cano (2006) MSTP Neuroscience Graduate Program
Angela Arensdorf (2008) Anatomy & Cell Biology Program
Cory Massey (2010) Neuroscience Graduate Program
Calvin Carter (2010) Neuroscience Graduate Program

Summer Undergraduate MSTP Research (SUMR)

Jeremy Sturgill (2006)
Christopher Gambino (2008)
Amanda Benavides (2008)
Tiffany Berkel (2010)

Secondary Student Training Program-Summer for High School Students (SSTP)

Kira Spencer (2005, 2006)
Ashwath Gunasekar (2007)

Research Assistants

Nicole Bode (2005-2014)
Adam Illif (2006-2007)

Undergraduate Lab Assistants

Dwiju Kumar
Nicolle Wolken
Nicole Bode
Katherine McLaughlin
Stephen Estelle

PubMed: Pedro Gonzalez-Alegre

2015

Edwards C, Leira E and Gonzalez-Alegre P. Failed lumbar puncture is more about obesity than ability. Neurology 2015;84(10):e69-72

2014

Noetzli L, Sanz P, Brodsky G, Hinckley J, Gonzalez-Alegre P and Di Paola J. A Novel Mutation in PLP1 Causes Severe Hereditary Spastic Paraplegia Type 2. Gene 2014;533(1):447-50.

García-Casares N, Berthier ML, Jorge RE, Gonzalez-Alegre P, Gutiérrez-Cardo A, Rioja-Villodres J, Acion L, Ariza-Corbo MJ, Nabrozidis A, García-Arnés JA, González-Santos P. Structural and functional brain changes in middle-aged type 2 diabetic patients: a cross-sectional study. J Alzheimers Dis 2014;40(2):375-86

Lamichhane D, Gonzalez-Alegre P, Narayanan N. Two cases of pregnancy in Parkinson’s disease. Parkinsonism & Relat Disord 2014;20(2):239-40.

Gonzalez-Alegre P, Schneider RL, Hoffman H. Clinical, etiological, and therapeutic features of jaw-opening and jaw-closing oromandibular dystonias: a decade of experience at a single treatment center. Tremor Other Hyperkinet Mov. 2014;4:231 (doi: 10.7916/D8TH8JSM)

García-Casares N, Jorge RE, García-Arnés JA, Acion L, Berthier ML, Gonzalez-Alegre P, Nabrozidis A, Gutiérrez A, Ariza MJ, Rioja J, González-Santos P. Cognitive Dysfunctions in Middle-Aged Type 2 Diabetic Patients and Neuroimaging Correlations: A Cross-Sectional Study. J Alzheimers Dis 2014;42(4):1337-46.

Gonzalez-Alegre P, Di Paola J, Wang K, Fabbro S, Yu HC, Shaikh TH, Darbro BW, Bassuk AG. Evaluating familial essential tremor with novel genetic approaches: is it a genotyping or phenotyping issue? Tremor Other Hyperkinet Mov 2014;20;4:258.

2013

Gonzalez-Alegre P, Buffard V, Wang K, Henien S and Morcuende J. Exploring the link between dystonia genes and idiopathic scoliosis. Journal of Pediatric Orthopaedics 2013;33(6):e65-6.

Paemka L, Mahajan VB, Skeie JM, Sowers LP, Ehaideb SN, Gonzalez-Alegre P, Sasaoka T, Tao H, Miyagi A, Ueno N, Takao K, Miyakawa T, Wu S, Darbro BW, Ferguson PJ, Pieper AA, Britt JK, Wemmie JA, Rudd DS, Wassink T, El-Shanti H, Mefford HC, Carvill GL, Manak JR and Bassuk AG. PRICKLE1 Interaction with SYNAPSIN I Reveals a Role in Autism Spectrum Disorders. PLOS One 2013;8(12):e80737

Letort D, Gonzalez-Alegre P. Huntington Disease in Children. Handbook of Neuropediatrics (edited by O. Dulac, H. Sarnat, M. Lassonde) Handbook of Clinical Neurology, 3rd series (edited by Aminoff, Boller and Swaab). Elsevier (2013)113:1913-7.

2012

Kakazu Y, Koh JY, Ho KW, Gonzalez-Alegre P, Harata NC. Synaptic vesicle recycling is enhanced by torsinA that harbors the DYT1 dystonia mutation. Synapse 2012;66(5):453-64

Onwuameze O, McCormick L, Gonzalez P, Beeghly J, Yasser K. Maintenance ECT in a Patient with Multiple System Atrophy and Bipolar Disorder. J ECT. 2012;28(2):e1-2.

Kakazu Y, Koh JY, Iwabuchi S, Gonzalez-Alegre P, Harata NC. Miniature release events of glutamate from hippocampal neurons are influenced by the dystonia-associated protein torsinA. Synapse 2012;66(9):807-22.

Beglinger LJ, Prest L, Mills JA, Paulsen JS, Smith MM, Gonzalez-Alegre P, Rowe KC, Nopoulos P and Uc EY. Clinical predictors of driving status in Huntington disease. Movement Disorders 2012;27(9):1146-52.

Bode N, Massey C, Gonzalez-Alegre P. DYT1 Knock-In Mice Are Not Sensitized against Mitochondrial Complex-II Inhibition. PLoS One. 2012;7(8):e42644.

Byars JA, Beglinger LJ, Moser DJ, Gonzalez-Alegre P, Nopoulos P. Substance abuse may be a risk factor for earlier onset of Huntington disease. J Neurol 2012 259(9):1824-31.

Gordon KL, Glenn KA, Bode N, Wen HM, Paulson HL, and Gonzalez-Alegre P. The ubiquitin ligase FBG1 promotes the degradation of the disease-linked protein torsinA through the ubiquitin proteasome pathway and macroautophagy. Neuroscience 2012;224C:160-171.

2011

Gordon KL, Glenn KA, Gonzalez-Alegre P. Exploring the influence of torsinA expression on protein quality control. Neurochem Res 2011;36(3):452-9

Martin JN, Wolken N, Brown T, Dauer WT, Ehrlich ME, Gonzalez-Alegre P. Lethal toxicity caused by expression of shRNA in the mouse striatum: implications for therapeutic design. Gene Therapy 2011;18(7):666-73

Maric M, Shao J, Ryan R, Wong CS, Gonzalez-Alegre P, Roller R. A functional role for torsinA in herpes simplex virus type 1 nuclear egress. J Virology 2011;85(19):9667-79

Strader S, Rodnitzky R, Gonzalez-Alegre P. Secondary dystonia in a botulinum toxin clinic: clinical characteristics, neuroanatomical substrate and comparison with idiopathic dystonia. Parkinsonism & Relat Disord 2011;17(10):749-52.

2010

Page ME, Bao L, Andre P, Pelta-Heller J, Gonzalez-Alegre P, Bogush A, Khan LE, Iacovitti L. Rice ME, Ehrlich ME. Cell-autonomous alteration of dopaminergic transmission by wild type and mutant (ΔE) TorsinA in transgenic mice. Neurobiology of Disease 2010;39:318-326.

Harris GM, Dodelzon K, Gong L, Gonzalez-Alegre P, Paulson HL. Splice isoforms of ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLOS One 2010;5(10):e13695

Wen HM, Kim N, Mallinger A, Gonzalez-Alegre P and Glenn KA. A promiscuous ubiquitin ligase controls cell cycle through a unique mechanism. Cell Cycle 2010;9(22).

Gonzalez-Alegre P. RNA interference (2010). In: Kompoliti K, and Verhagen Metman L (eds.) Encyclopedia of Movement Disorders. Oxford: Academic Press.

Gonzalez-Alegre P. Descriptions of cervical dystonia by Sir Charles Bell. Mov Disord 2010;25(3):257-9.

2010

Harlow T, Gonzalez-Alegre P. High prevalence of reported tremor in Klinefelter syndrome. Parkinsonism & Relat Disord 2009;15(5):393-5

Martin JN, Bair TB, Bode N, Dauer WT, Gonzalez-Alegre P. Transcriptional and proteomic profiling in a cellular model of DYT1 dystonia. Neuroscience 2009;164(2):563-72.

Harlow T, Rodnitzky R, Gonzalez-Alegre P. From essential tremor to Klinefelter through fragile X, an unexpected journey. Mov Disord 2008;23(9):1328-1329.

Gordon KL, Gonzalez-Alegre P. Consequences of the DYT1 mutation on torsinA oligomerization and degradation. Neuroscience 2008;157:588-595.

Bassuk AG, Wallace RH, Buhr A, Buller A, Shimojo M, Miyata S, Chen S, Gonzalez-Alegre P, Griesbach HL, Shu W, Nashelsky M, Vladar EK, Antic D, Ferguson PJ, Cirak S, Voit T, Scott MP, Axelrod JD, Gurnett G, Daoud AS, Afawi Z, Neufeld MY, Korczyn AD, Kivity S, Mazarib A, Straussberg R, Walid S, Slusarski DC, Berkovic SF, El-Shanti HI. A homozygous mutation in human PRICKLE1 causes an autosomal-recessive progressive myoclonus epilepsy-ataxia syndrome. Am J Human Genet 2008;83(5):572-81

Harper SQ, Gonzalez-Alegre P. Lentivirus-mediated RNA interference in mammalian neurons. Methods Mol Biol 2008;442:95-112.

Gonzalez-Alegre P. Monomelic Parkinsoniam Tremor Caused by Contralateral Substantia Nigra Stroke. Parkinsonism & Relat Disord. 2007;13(3):182-184.

Eddey GE, Puig JG, Reich SG. Delineation of the Motor Disorder of Lesch-Nyhan Disease. Brain 2006;129:1201-17

Gonzalez-Alegre P, Kelkar P, Rodnitzky RL. Isolated high frequency jaw tremor relieved by botulinum toxin injections. Mov Disord 2006;21(7):1049-50.

Gonzalez-Alegre P. Severe interscapular pain and elevated creatine kinase: the answer was in the ankles. Emerg Med J 2005;22:148-155.

Gonzalez-Alegre P, Bode N, Davidson BL, Paulson HP. Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1. J Neurosc 2005;25: 10502-10509.

Gonzalez-Alegre P, Paulson HP. Aberrant cellular behavior of mutant TorsinA implicates nuclear envelope dysfunction in DYT1 dystonia. J Neurosci 2004;24(11):2593-2601.

Gonzalez-Alegre P, Tippin J. Prolonged cortical electrical depression and diffuse vasospasm without ischemia in a case of severe hemiplegic migraine during pregnancy. Headache 2003;43:72-75.

Gonzalez-Alegre P, Miller VM, Davidson B, Paulson H. Toward therapy for DYT1 dystonia: Allele-specific silencing of mutant torsinA. Annals of Neurology 2003;53:781-787.

Gonzalez-Alegre P, Ammache Z, Davis PH, Rodnitzky R. Moyamoya-induced paroxysmal dyskinesia. Mov Disord 2003;18(9):1051-1056.

Bonansco C, González de la Vega A , González-Alegre P, Borde M, García-Segura LM and Buño W. Tetanic stimulation of Schaffer collaterals induces rhythmic bursts via NMDA receptor activation in rat CA1 pyramidal neurons. Hippocampus 2002;12:434-446

Gonzalez-Alegre P, Recober A, Kelkar P. Idiopathic Brachial Neuritis. Iowa Orthop J 2002; 22:81-85.

Gonzalez-Alegre P, Afifi AK. Clinical Characteristics of Childhood-Onset (Juvenile) Huntington's Disease. Report of twelve patients and review of the literature. J Child Neurol 2006;21(3):223-229.

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