Movement Disorders book cover

Movement Disorders

Genetics and Models

The use of animal models is a key aspect of scientific research in numerous fields of medicine. Movement Disorders, Second Edition vigorously examines the important contributions and application of animal models to the understanding of human movement disorders, and serves as an essential resource for basic neuroscientists engaged in movement disorders research. Academic clinicians, translational researchers and basic scientists are brought together to connect experimental findings made in different animal models to the clinical features, pathophysiology and treatment of human movement disorders.

A vital feature of this book is an ancillary website with video clips of human movement disorders and their corresponding animal models. The book is divided into sections on Parkinson's disease, Huntington's disease, dystonia, tremor, paroxysmal movement disorders, ataxia, myoclonus, restless legs syndrome, drug-induced movement disorders, multiple system atrophy, progressive supranuclear palsy/corticobasal degeneration, and spasticity. This book serves as an essential resource for both clinicians interested in the science being generated with animal models and basic scientists studying the pathogenesis of particular movement disorders.

General neurologists, neurologists with subspecialty interest in movement disorders, neuroscientists, rehabilitation physicians, psychiatrists, and academic veterinarians with an interest in animal models.

Hardbound, 1258 Pages

Published: October 2014

Imprint: Academic Press

ISBN: 978-0-12-405195-9


  • "The book is a timely review of animal models of movement disorders...The text is an excellent resource for neurologists and researchers specializing in movement disorders. In addition, this book will undoubtedly be a useful reference source for general neurologists, neurology residents, and basic science fellow and students." - Zbigniew K. Wszolek, Department of Neurology, Mayo Clinic Jacksonville in PARKINSONISM AND RELATED DISORDERS (September 2005) "...this new textbook provides a comprehensive treatment of animal models used to study human movement disorders and should serve the neurologist, researcher, and other interested scientists very well." --Dr. A.S. Fix, Central Product Safety, The Proctor and Gamble Co, Miami Valley Innovation Center, Cincinnati, OH, in VETERINARY PATHOLOGY (43:5) 2006


  • Section I. Scientific Foundations
    1. Taxonomy and Clinical Features of Movement Disorders
        Mark Stacy and Patrick Hickey
    2. Modeling Disorders of Movement
        Mark S. LeDoux
    3. New Transgenic Technologies
        Thomas L. Saunders
    4. Assessment of Movement Disorders in Rodents
        H. A. Jinnah and E. J. Hess
    5. Drosophila
        Rami R. Ajjuri, Marleshia Hall, Lawrence T. Reiter and Janis M. O'Donnell
    6. Use of C. elegans to Model Human Movement Disorders
        Guy A. Caldwell and Kim A. Caldwell
    7. Zebrafish
        Edward A. Burton
    8. Techniques for Motor Assessment in Rodents
        Amandeep Mann and Marie- Francoise Chesselet
    9. Induced Pluripotent Stem Cells (iPSCs) to Study and Treat Movement Disorders
        Devkanya Dutta and Kwang-Soo Kim
    10. Neurophysiological Assessment of Movement Disorders in Humans
          Nicolás M. Phielipp and Robert Chen
    11. Neurophysiological and Optogenetic Assessment of Brain Networks Involved in Motor Control
          Ilse S. Pienaar, David Trevor Dexter and Viviana Gradinaru
    12. Functional Imaging to Study Movement Disorders
          Wataru Sako, Aziz M. Uluğ and David Eidelberg
    13. Human and Non-Human Primate Neurophysiology to Understand the Pathophysiology of Movement Disorders
          Nicole Swann and Philip Starr

    Section II.  Parkinson Disease
    14. The Phenotypic Spectrum of Parkinson Disease
          Ronald F. Pfeiffer
    15. Genetics and Molecular Biology of Parkinson Disease
          Simona Petrucci, Giuseppe Arena and Enza Maria Valente
    16. Genotype-Phenotype Correlations in Parkinson Disease
          Andreas Puschmann and Zbigniew K. Wszolek
    17. From  Man to Mouse: The MPTP Model of Parkinson Disease
          Vernice Jackson Lewis, Deranda Lester, Elena Kozina, Serge Przedborski and Richard Jay Smeyne
    18. Rodent Models of Autosomal Dominant Parkinson Disease
          Jianfeng Xiao, Satya Vemula and Zhenyu Yue
    19. Rodent Models of Autosomal Recessive Parkinson Disease
          Suzana Gispert, Georg Auburger, Korah P. Kuruvilla and Mark S. LeDoux
    20. Drosophila models of PD
          Brian Ernest Staveley
    21. Primates Models of PD-Treatment Related Complications
          Susan Fox, Jonathan Brotchie and Tom M. Johnston
    22. Rodent Models of Treatment-Related Complications in Parkinson disease
          Veronica Francardo, Hanna Iderberg, Hanna Lindgren and M. Angela Cenci
    23. Methods and models of the non-motor symptoms of Parkinson disease
          Michael P. McDonald

    Section III.  Dystonia
    24. Dystonia: Phenotypes and Genetics
          Mark S. LeDoux
    25. Murine Models of Caytaxin Deficiency
          Mark S. LeDoux
    26. Animal Models of Focal Dystonia
          L. Craig Evinger
    27. Mouse Models of Dystonia
          E. J. Hess and H. A. Jinnah
    28. Rodent Models of Autosomal Dominant Primary Dystonia
          Janneth Oleas, Fumiaki Yokoi, Mark P. DeAndrade and Yuqing Li
    29. Modeling Dystonia-Parkinsonism
          Kamran Khodakhah and Diany Paola Calderon

    Section IV.  Huntington Disease
    30. Genetics of Huntington Disease (HD),  HD-Like Disorders, and Other Choreiform Disorders
          Ruth Walker
    31. Murine Models of HD
          Gelareh Mazarei and Blair R. Leavitt
    32. Use of Genetically Engineered Mice to Study the Biology of Huntington
          Paula Dietrich and Ioannis Dragatsis
    33. Modelling Huntington's disease in yeast and invertebrates
          Robert P. Mason, Carlo Breda, Gurdeep S. Kooner, Giovanna R. Mallucci, Charalambos P. Kyriacou and Flaviano Giorgini
    34. HDL2 Mouse
          Dobrila D. Rudnicki, X. William Yang and Russell L. Margolis
    35. Analysis of Non-Motor Features in Murine Models of HD
          Barbara Baldo and Asa Petersen

    Section V.  Tremor
    36. Essential Tremor
          Jay A. Van Gerpen and Owen A. Ross
    37.  Use of the Harmaline and α1 Knockout Models to Identify  Molecular Targets for Essential Tremor
          Adrian Handforth
    38. Physiological and Behavioral Assessment of Tremor in Rodents
          John Salamone, Samantha Podurgiel, Lyndsey E. Collins-Praino and Mercè Correa
    39. Mouse Models of the Fragile X tremor/ataxia Syndrome (FXTAS)  and the Fragile X Premutation
          Robert F. Berman, Michael Ryan Hunsaker and Jared Schwartzer

    Section VI.  Myoclonus
    40. Myoclonus: Classification, Clinical Features and Genetics
          Steven Frucht and Amar Patel
    41. Mouse Model of Unverricht-Lundborg Disease
          Outi Kopra, Tarja Joensuu and Anna-Elina Lehesjoki
    42. Rat Model of Posthypoxic Myoclonus
          Kwok-Keung Tai and Daniel Truong
    43. Generating Mouse Models of Mitochondrial Disease
          Emil Ylikallio and Henna Tyynismaa

    Section VII.  Tics
    44. Tics and Tourette Syndrome: Phenomenology
          Neepa Patel and Joseph Jankovic
    45. Genetics of Tourette Syndrome
          Peristera Paschou, Pieter J. Hoekstra and Gary A. Heiman
    46. Neural Circuit Abnormalities in Tourette Syndrome
          Irene Neuner
    47. Animal models of Tourette syndrome and obsessive-compulsive disorder
          Christopher Pittenger

    Section VIII.  Paroxysmal Movement Disorders
    48. Paroxysmal Movement Disorders: Clinical and Genetic Features
          Emmanuel Roze, Aurélie Méneret and Marie Vidailhet
    49. Mouse Models of PNKD
          Korah P. Kuruvilla and Mark S. LeDoux
    50. GLUT1 Deficiency (G1D)
          Juan M. Pascual
    51. Animal Models of Episodic Ataxia Type 1 (EA1)
          Mauro Pessia, Maria Cristina D'Adamo and Giuseppe Di Giovanni
    52. Mouse Models of Episodic Ataxia Type 2
          Samuel J. Rose and E. J. Hess

    Section IX.  Tauopathies
    53. Tauopathies: Classification, Clinical Features and Genetics
          Keith A. Josephs
    54. Drosophila Models of Tauopathy
          Yousuf Ali, Kai Ruan and R. Grace Zhai
    55. Tauopathy mouse models
          Yan Ren, Naruhiko Sahara, Benoit Giasson and Jada Lewis
    56. Tau protein: Biology and Pathobiology
          Nicholas M. Kanaan, Diana S. Himmelstein, Sarah M. Ward, Benjamin Combs and Lester Irvin Binder

    Section X.  Other Parkinsonian Syndromes: NBIA, MSA, PD+spasticity, PD+dystonia
    57. Clinical Phenomenology and Genetics of other Parkinsonian Syndromes Associated with either Dystonia or Spasticity
          Susanne Schneider
    58. Animal models of multiple system atrophy
          Florian Krismer, Daniel Kuzdas, Carlo Colosimo, Nadia Stefanova and Gregor K. Wenning
    59. Modeling PKAN in Mice and Flies
          Ody Sibon, Susan Hayflick and Valeria Tiranti
    60. Mouse models of FA2H deficiency
          Kathleen A. Willet and Hiroko Hama
    61. Mouse models of Neuroaxonal Dystrophy caused by PLA2G6 Gene Mutations
          Devika P. Bagchi and Paul Kotzbauer

    Section XI.  Ataxias
    62. Genetics and Clinical Features of Inherited Ataxias
          S.H. Subramony and T. Ashizawa
    63. Animal Models of Spinocerebellar  Ataxia Type 1 (SCA1)
          Puneet Opal and Harry T. Orr
    64. Mouse Models of SCA3 and Other Polyglutamine Repeat Ataxias
          Maciej Figiel, Wlodzimierz J. Krzyzosiak, Pawel M. Switonski and Wojciech J. Szlachcic
    65. Animal Models of Friedreich Ataxia
          Massimo Pandolfo
    66. Ataxia-Telangiectasia and the Biology of ATM
          Karl Herrup, Jianmin Chen, Jiali Li and Mark Plummer
    67. Autosomal Recessive Ataxias due to Defects in DNA Repair
          Sherif F. El-Khamisy and Owen Spencer Wells
    68. C. elegans models to study the molecular biology of ataxias
          Pilar Gonzalez-Cabo and Rafael P. Vazquez-Manrique

    Section XII.  Hereditary Spastic Paraplegia
    69. Hereditary Spastic Paraplegias:  Genetics and Clinical Features
          Benoît Renvoisé and Craig Blackstone
    70. Mouse models of autosomal dominant spastic paraplegia
          Coralie Fassier, Jamilé Hazan and Judith Melki
    71. Murine Models of Autosomal Recessive Hereditary Spastic Paraplegia
          Craig Blackstone
    72. Modeling HSP in Zebrafish
          João M. Peres and Corinne Houart
    73. Drosophila Models of Hereditary Spastic Paraplegia
          Emily F. Ozdowski, Sally L. Baxter and Nina Tang Sherwood
    74. Caenorhabditis elegans Models of Hereditary Spastic Paraplegia
          Peter Hedera
    75. Use of Arabidopsis to model hereditary spastic paraplegia and other movement disorders
          John Gardiner

    Section XIII.  Restless Legs Syndrome
    76. Clinical Phenotype and Genetics of Restless Legs Syndrome
          Eva C. Schulte and Juliane Winkelmann
    77. Combined D3 Receptor/ Iron Deficient Mouse Model
          David Liebetanz and Florian Klinker
    78. Use of Drosophila to Study Restless Legs Syndrome
          Subhabrata Sanyal
    79. The A11 Lesion/Iron Deprivation Animal Model of Restless Legs Syndrome
          William Ondo
    80. Btbd9 Knockout Mice as a Model of Restless Legs Syndrome
          Mark P. DeAndrade and Yuqing Li



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