2012
DOI: 10.1007/s00335-011-9382-y
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Abstract: Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder in which the loss of dystrophin causes progressive degeneration of skeletal and cardiac muscle. Potential therapies that carry substantial risk, such as gene and cell-based approaches, must first be tested in animal models, notably the mdx mouse and several dystrophin-deficient breeds of dogs, including golden retriever muscular dystrophy (GRMD). Affected dogs have a more severe phenotype, in keeping with that of DMD, so may better predict dis… Show more

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Cited by 148 publications
(179 citation statements)
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“…2012). Expression of OPN in the severely affected VL and LDE and the mildly affected CS of GRMD dogs (Nghiem et al .…”
Section: Resultsmentioning
confidence: 99%
“…2012). Expression of OPN in the severely affected VL and LDE and the mildly affected CS of GRMD dogs (Nghiem et al .…”
Section: Resultsmentioning
confidence: 99%
“…This disorder usually results in an absence of dystrophin and severe muscular degeneration, but rarely a mutation can lead to a structurally altered and partially functional dystrophin protein resulting in a less severe phenotype 14, 15, 18. Dystrophin‐deficient CXLMD has been reported in 1 male Labrador Retriever puppy1 and has been extensively investigated in Golden Retrievers 19. Most puppies with dystrophin‐deficient CXLMD show muscular weakness as soon as they begin to ambulate.…”
Section: Discussionmentioning
confidence: 99%
“…Clinical weakness and gait abnormalities are the first manifestations in puppies affected by DMD at 6 to 10 weeks old, similar to that observed in DMD children who exhibit their first clinical signs at 2 to 4 years old (VALENTINE et al, 1988). A notable feature in DGR dogs is the enlargement of the base of the tongue due to hypertrophy and muscle pseudohypertrophy, which leads to pharyngeal and esophageal dysfunction, resulting in dysphagia, salivation, regurgitation, and finally, aspiration pneumonia (VALENTINE et al, 1988;NAKAMURA & TAKEDA, 2011;KORNEGAY et al, 2012). Progressive gait impairment is evidenced in DGR dogs by the sixth week of age as a result of atrophy of trunk and temporalis muscles, plantar posture due to overextension at the carpus, overflexion at the tarsus and abduction of the paws, as well as progressive lumbar kyphosis and consequent lordosis (VALENTINE et al, 1988;SHELTON & ENGVALL, 2005;NAKAMURA & TAKEDA, 2011;KORNEGAY et al, 2012).…”
Section: Clinical Aspects Of the Disease In The Dgr Dogmentioning
confidence: 52%
“…Both DMD and DGR present progressive clinical signs and severe myopathy with initial fiber necrosis and regeneration associated with connective tissue proliferation in the endomysium and perimysium (VALENTINE et al, 1990,;MIJAZATO et al, 2011b;KORNEGAY et al, 2012). Also, adult dogs have a body mass compatible with that of patients with DMD.…”
Section: Dog As Model Of Muscular Dystrophymentioning
confidence: 99%