Animal Models of Autosomal Recessive Parkinsonism.

Biomedicines
Guendalina BastioliJenny Sassone

Abstract

Parkinson's disease (PD) is the most common neurodegenerative movement disorder. The neuropathological hallmark of the disease is the loss of dopamine neurons of the substantia nigra pars compacta. The clinical manifestations of PD are bradykinesia, rigidity, resting tremors and postural instability. PD patients often display non-motor symptoms such as depression, anxiety, weakness, sleep disturbances and cognitive disorders. Although, in 90% of cases, PD has a sporadic onset of unknown etiology, highly penetrant rare genetic mutations in many genes have been linked with typical familial PD. Understanding the mechanisms behind the DA neuron death in these Mendelian forms may help to illuminate the pathogenesis of DA neuron degeneration in the more common forms of PD. A key step in the identification of the molecular pathways underlying DA neuron death, and in the development of therapeutic strategies, is the creation and characterization of animal models that faithfully recapitulate the human disease. In this review, we outline the current status of PD modeling using mouse, rat and non-mammalian models, focusing on animal models for autosomal recessive PD.

References

Jul 1, 1990·The Journal of Cell Biology·S Ahle, E Ungewickell
May 1, 1994·Acta Neurologica Scandinavica·A S Najim al-DinM Hiari
Jan 25, 1996·Nature·P S McPhersonP De Camilli
Sep 10, 1998·Biochemical and Biophysical Research Communications·N HattoriY Mizuno
May 20, 2000·Mammalian Genome : Official Journal of the International Mammalian Genome Society·T KitadaN Shimizu
Jul 20, 2001·American Journal of Human Genetics·C M van DuijnP Heutink
Mar 19, 2003·Proceedings of the National Academy of Sciences of the United States of America·Jessica C GreeneLeo J Pallanck
Apr 1, 2003·Neuromolecular Medicine·Mark R Cookson
Aug 14, 2003·Human Molecular Genetics·Jean-Michel ItierJusto Garcia de Yebenes
Aug 22, 2003·The Journal of Biological Chemistry·Matthew S GoldbergJie Shen
Sep 4, 2003·Annals of Neurology·Patrick M Abou-SleimanNicholas W Wood
Oct 2, 2003·Human Molecular Genetics·Mark R CooksonMatthew J Farrer
Feb 21, 2004·Human Molecular Genetics·Ignacio F MataMatthew J Farrer
Feb 27, 2004·The Journal of Biological Chemistry·James J PalacinoJie Shen
Mar 12, 2004·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Diego LorenzettiMonica Justice
Apr 17, 2004·Science·Enza Maria ValenteNicholas W Wood
Jul 14, 2004·Proceedings of the National Academy of Sciences of the United States of America·Rainer Von CoellnTed M Dawson
Aug 25, 2004·Lancet·Nobutaka Hattori, Yoshikuni Mizuno
Feb 3, 2005·Proceedings of the National Academy of Sciences of the United States of America·Francisco A Perez, Richard D Palmiter
Mar 24, 2005·Proceedings of the National Academy of Sciences of the United States of America·Raymond H KimTak W Mak
Apr 1, 2005·The Journal of Biological Chemistry·Linan ChenXiaoxi Zhuang
May 25, 2005·Proceedings of the National Academy of Sciences of the United States of America·Alexander J WhitworthLeo J Pallanck
Jul 9, 2005·Proceedings of the National Academy of Sciences of the United States of America·Guang-Ho ChaKyoung Sang Cho
Jul 22, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Joseph M SavittTed M Dawson
Sep 6, 2005·Current Biology : CB·Fiona M MenziesKyung-Tai Min
Oct 7, 2005·Human Molecular Genetics·Laura SilvestriGiorgio Casari
Jul 5, 2006·Proceedings of the National Academy of Sciences of the United States of America·Yufeng YangBingwei Lu
Aug 26, 2006·Nature Clinical Practice. Neurology·Christine Klein, Michael G Schlossmacher
Aug 31, 2006·Journal of Neuroscience Research·Shigeto SatoNobutaka Hattori
Dec 6, 2006·Genesis : the Journal of Genetics and Development·Sandra GoebbelsKlaus-Armin Nave
Dec 13, 2006·Genome Biology·Yoshihito TaniguchiEdwin Cuppen
Dec 15, 2006·Journal of Neurochemistry·Sandrine BretaudOliver Bandmann
Feb 3, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Tzu-Kang SangGeorge R Jackson
Mar 29, 2007·International Journal of Biological Sciences·Hongxia ZhouXu Gang Xia
Apr 7, 2007·Human Molecular Genetics·Christine C StichelHermann Lübbert

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