title: Animal models of muscular dystrophy.
abstract: The muscular dystrophies (MDs) represent a diverse collection of inherited human disorders, which affect to varying degrees skeletal, cardiac, and sometimes smooth muscle (Emery, 2002). To date, more than 50 different genes have been implicated as causing one or more types of MD (Bansal et al., 2003). In many cases, invaluable insights into disease mechanisms, structure and function of gene products, and approaches for therapeutic interventions have benefited from the study of animal models of the different MDs (Arnett et al., 2009). The large number of genes that are associated with MD and the tremendous number of animal models that have been developed preclude a complete discussion of each in the context of this review. However, we summarize here a number of the more commonly used models together with a mixture of different types of gene and MD, which serves to give a general overview of the value of animal models of MD for research and therapeutic development.
authors.affiliation: Division of Medical Genetics, Department of Neurology, University of Washington, Seattle, Washington, USA.
authors.forename: Rainer
authors.initials: R
authors.lastname: Ng
authors.forename: Glen B
authors.initials: GB
authors.lastname: Banks
authors.forename: John K
authors.initials: JK
authors.lastname: Hall
authors.forename: Lindsey A
authors.initials: LA
authors.lastname: Muir
authors.forename: Julian N
authors.initials: JN
authors.lastname: Ramos
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Wicki
authors.forename: Guy L
authors.initials: GL
authors.lastname: Odom
authors.forename: Patryk
authors.initials: P
authors.lastname: Konieczny
authors.forename: Jane
authors.initials: J
authors.lastname: Seto
authors.forename: Joel R
authors.initials: JR
authors.lastname: Chamberlain
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Chamberlain
mesh_terms: D000818:Animals; D002118:Calcium / Q000378:metabolism; D004195:Disease Models, Animal; D006801:Humans; D009124:Muscle Proteins / Q000737:chemistry / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism / Q000473:pathology*
chemical_list: D009124:Muscle Proteins; D002118:Calcium