RGD Reference Report - Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy.

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Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy.
Authors:	Larcher, Thibaut Lafoux, Aude Tesson, Laurent Remy, Séverine Thepenier, Virginie François, Virginie Le Guiner, Caroline Goubin, Helicia Dutilleul, Maéva Guigand, Lydie Toumaniantz, Gilles De Cian, Anne Boix, Charlotte Renaud, Jean-Baptiste Cherel, Yan Giovannangeli, Carine Concordet, Jean-Paul Anegon, Ignacio Huchet, Corinne
Citation:	Larcher T, etal., PLoS One. 2014 Oct 13;9(10):e110371. doi: 10.1371/journal.pone.0110371. eCollection 2014.
RGD ID:	12880034
Pubmed:	PMID:25310701 (View Abstract at PubMed)
PMCID:	PMC4195719 (View Article at PubMed Central)
DOI:	DOI:10.1371/journal.pone.0110371 (Journal Full-text)
A few animal models of Duchenne muscular dystrophy (DMD) are available, large ones such as pigs or dogs being expensive and difficult to handle. Mdx (X-linked muscular dystrophy) mice only partially mimic the human disease, with limited chronic muscular lesions and muscle weakness. Their small size also imposes limitations on analyses. A rat model could represent a useful alternative since rats are small animals but 10 times bigger than mice and could better reflect the lesions and functional abnormalities observed in DMD patients. Two lines of Dmd mutated-rats (Dmdmdx) were generated using TALENs targeting exon 23. Muscles of animals of both lines showed undetectable levels of dystrophin by western blot and less than 5% of dystrophin positive fibers by immunohistochemistry. At 3 months, limb and diaphragm muscles from Dmdmdx rats displayed severe necrosis and regeneration. At 7 months, these muscles also showed severe fibrosis and some adipose tissue infiltration. Dmdmdx rats showed significant reduction in muscle strength and a decrease in spontaneous motor activity. Furthermore, heart morphology was indicative of dilated cardiomyopathy associated histologically with necrotic and fibrotic changes. Echocardiography showed significant concentric remodeling and alteration of diastolic function. In conclusion, Dmdmdx rats represent a new faithful small animal model of DMD.

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Disease Annotations

Cardiac Fibrosis (IMP,ISO)

dilated cardiomyopathy (IMP,ISO)

Duchenne muscular dystrophy (IMP,ISO)

Phenotype Annotations

Mammalian Phenotype

abnormal heart echocardiography feature (IMP)

decreased body length (IMP)

decreased body mass index (IMP)

decreased body weight (IMP)

decreased heart weight (IMP)

decreased locomotor activity (IMP)

decreased skeletal muscle fiber diameter (IMP)

decreased tibialis anterior weight (IMP)

increased circulating creatine kinase level (IMP)

muscle weakness (IMP)

Objects Annotated

Genes (Rattus norvegicus)

Dmd (dystrophin)

Dmd^em1Ang (dystrophin; TALEN-induced mutant1, Ang)

Genes (Mus musculus)

Dmd (dystrophin, muscular dystrophy)

Genes (Homo sapiens)

DMD (dystrophin)

Strains

SD-Dmd^em1Ang (NA)

Additional Information

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Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy.

Mammalian Phenotype