Literature DB >> 29067659

Probing the Pathogenesis of Duchenne Muscular Dystrophy Using Mouse Models.

Alexander Morrison-Nozik1, Saptarsi M Haldar2,3.   

Abstract

Investigations using mouse models have provided seminal insights into the pathogenesis of Duchenne muscular dystrophy and the development of novel therapeutics. Several important methods have been considered standard-in-the-field for analyses of skeletal muscle weakness, strength, endurance, and histopathology, as well as responses to therapeutics such as glucocorticoids, disease modifying drugs which are part of the current standard of care for patients with this disease. Here we describe optimized genetic, genomic, and physiologic assays to probe dystrophic pathobiology in the mdx mouse and related strains.

Entities:  

Keywords:  Duchenne; Exercise; Glucocorticoids; Muscular dystrophy; Skeletal muscle; mdx

Mesh:

Substances:

Year:  2018        PMID: 29067659      PMCID: PMC5805135          DOI: 10.1007/978-1-4939-7374-3_8

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN: 1064-3745


  33 in total

1.  Drug screening in a zebrafish model of Duchenne muscular dystrophy.

Authors:  Genri Kawahara; Jeremy A Karpf; Jennifer A Myers; Matthew S Alexander; Jeffrey R Guyon; Louis M Kunkel
Journal:  Proc Natl Acad Sci U S A       Date:  2011-03-14       Impact factor: 11.205

2.  Muscular dystrophy in a litter of golden retriever dogs.

Authors:  J N Kornegay; S M Tuler; D M Miller; D C Levesque
Journal:  Muscle Nerve       Date:  1988-10       Impact factor: 3.217

3.  Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin alpha2-deficient mice.

Authors:  A M Connolly; R M Keeling; S Mehta; A Pestronk; J R Sanes
Journal:  Neuromuscul Disord       Date:  2001-11       Impact factor: 4.296

4.  Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy.

Authors:  A E Deconinck; J A Rafael; J A Skinner; S C Brown; A C Potter; L Metzinger; D J Watt; J G Dickson; J M Tinsley; K E Davies
Journal:  Cell       Date:  1997-08-22       Impact factor: 41.582

5.  Evans Blue Dye as an in vivo marker of myofibre damage: optimising parameters for detecting initial myofibre membrane permeability.

Authors:  P W Hamer; J M McGeachie; M J Davies; M D Grounds
Journal:  J Anat       Date:  2002-01       Impact factor: 2.610

6.  Strength and corticosteroid responsiveness of mdx mice is unchanged by RAG2 gene knockout.

Authors:  Paul T Golumbek; Richard M Keeling; Anne M Connolly
Journal:  Neuromuscul Disord       Date:  2007-04-23       Impact factor: 4.296

Review 7.  RNA-Seq: a revolutionary tool for transcriptomics.

Authors:  Zhong Wang; Mark Gerstein; Michael Snyder
Journal:  Nat Rev Genet       Date:  2009-01       Impact factor: 53.242

Review 8.  Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy.

Authors:  Joe W McGreevy; Chady H Hakim; Mark A McIntosh; Dongsheng Duan
Journal:  Dis Model Mech       Date:  2015-03       Impact factor: 5.758

9.  Regulation of muscle fiber type and running endurance by PPARdelta.

Authors:  Yong-Xu Wang; Chun-Li Zhang; Ruth T Yu; Helen K Cho; Michael C Nelson; Corinne R Bayuga-Ocampo; Jungyeob Ham; Heonjoong Kang; Ronald M Evans
Journal:  PLoS Biol       Date:  2004-08-24       Impact factor: 8.029

Review 10.  The role of corticosteroids in muscular dystrophy: a critical appraisal.

Authors:  Corrado Angelini
Journal:  Muscle Nerve       Date:  2007-10       Impact factor: 3.217

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