Muscular dystrophy in a litter of golden retriever dogs

Kornegay, Joe N.; Tuler, S. M.; Miller, Doris M.; Levesque, Donald

doi:10.1002/mus.880111008

Cited by 209 publications

(158 citation statements)

References 20 publications

(2 reference statements)

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“…This may be due to improved survival of fibres reconstituted with transplanted cells but also to factors such as insulin-like growth factor 1, basic fibroblast growth factor and others released by donor cells 15 and contributing to fibre survival. At the end of treatment, the level of creatine kinase in all treated dogs was well below the those reported for this form of muscular dystrophy 9 . Other enzymes released by damaged muscle fibres, such as aspartate aminotransferase, showed an overall similar pattern (data not shown).…”

Section: Articlescontrasting

confidence: 57%

Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs

Sampaolesi

Blot

D’Antona

et al. 2006

Nature

674

569

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Duchenne muscular dystrophy remains an untreatable genetic disease that severely limits motility and life expectancy in affected children. The only animal model specifically reproducing the alterations in the dystrophin gene and the full spectrum of human pathology is the golden retriever dog model. Affected animals present a single mutation in intron 6, resulting in complete absence of the dystrophin protein, and early and severe muscle degeneration with nearly complete loss of motility and walking ability. Death usually occurs at about 1 year of age as a result of failure of respiratory muscles. Here we report that intra-arterial delivery of wild-type canine mesoangioblasts (vessel-associated stem cells) results in an extensive recovery of dystrophin expression, normal muscle morphology and function (confirmed by measurement of contraction force on single fibres). The outcome is a remarkable clinical amelioration and preservation of active motility. These data qualify mesoangioblasts as candidates for future stem cell therapy for Duchenne patients.

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Section: Articlescontrasting

confidence: 57%

Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs

Sampaolesi

Blot

D’Antona

et al. 2006

Nature

674

569

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“…Canine X-linked muscular dystrophy (CXMD) which has a splice site mutation in the dystrophin gene has been recognized and best characterized in the golden retriever breed in which it occurs spontaneously. Moreover, the signs and symptoms of canine disorder are similar to those of human DMD [8,16].…”

Section: Introductionmentioning

confidence: 72%

Canine X-Linked Muscular Dystrophy in Japan (CXMDJ).

Shimatsu¹,

Katagiri²,

Furuta³

et al. 2003

Exp. Anim.

Self Cite

115

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Abstract:The purpose of this study was to develop a strain of canine X-linked muscular dystrophy (CXMD), a model of Duchenne muscular dystrophy, in Japan. A female beagle was artificially inseminated with frozen-thawed spermatozoa derived from an affected golden retriever. Subsequently, two carrier female dogs (G1 carriers) and four normal male littermates were produced. Thereafter, the two G1 carriers were mated with beagle sires. As a result, each bitch whelped three times, and out of 54 pups, 17 affected male descendants, and 11 carrier female descendants (G2 carriers) were detected. One G2 carrier was then mated with a beagle sire and 15 pups in two whelpings were produced, including five affected males and four carrier females (G3 carriers). A total of 10 female beagles were artificially inseminated to evaluate the fertility of the frozen-thawed spermatozoa from the two affected dogs. The whelping rates of the two affected dogs were 4/5 and the litter sizes were 5.0 ± 1.41 and 6.0 ± 0.82, respectively. These results indicate that a canine X-linked muscular dystrophy colony has been established in Japan. We called them CXMD J .

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“…47,48 Golden retriever crossbred dogs from a GRMD colony maintained in the Boisbonne Center for Gene Therapy of Oniris, NantesAtlantic College of Veterinary Medicine, Food Sciences and Engineering were studied. Affected dogs, which have progressive clinical dysfunction similar to that of DMD boys, as previously described, 45,49 were initially identified based on PCR-based genotyping, and the pathology confirmed by a dramatic elevation of serum creatine kinase. 50 The animal experiments were approved by the French National Institute for Agronomic Research and were performed according to the guidelines of the Institute.…”

Section: Animalsmentioning

confidence: 99%

Systemic Delivery of Allogenic Muscle Stem Cells Induces Long-Term Muscle Repair and Clinical Efficacy in Duchenne Muscular Dystrophy Dogs

Rouger

Larcher

Dubreil

et al. 2011

The American Journal of Pathology

100

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Duchenne muscular dystrophy (DMD) is a genetic progressive muscle disease resulting from the lack of dystrophin and without effective treatment. Adult stem cell populations have given new impetus to cellbased therapy of neuromuscular diseases. One of them, muscle-derived stem cells, isolated based on delayed adhesion properties, contributes to injured muscle repair. However, these data were collected in dystrophic mice that exhibit a relatively mild tissue phenotype and clinical features of DMD patients. Here, we characterized canine delayed adherent stem cells and investigated the efficacy of their systemic delivery in the clinically relevant DMD animal model to assess potential therapeutic application in humans. Delayed adherent stem cells, named MuStem cells (muscle stem cells), were isolated from healthy dog muscle using a preplating technique. In vitro, MuStem cells displayed a large expansion capacity, an ability to proliferate in suspension, and a multilineage differentiation potential. Phenotypically, they corresponded to early myogenic progenitors and uncommitted cells. When injected in immunosuppressed dystrophic dogs, they contributed to myofiber regeneration, satellite cell replenishment, and dystrophin expression. Importantly, their systemic delivery resulted in long-term dystrophin expression, muscle damage course limitation with an increased regeneration activity and an interstitial expansion restriction, and persisting stabilization of the dog's clinical status. These results demonstrate that MuStem cells could provide an attractive therapeutic avenue for DMD patients.

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Muscular dystrophy in a litter of golden retriever dogs

Cited by 209 publications

References 20 publications

Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs

Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs

Canine X-Linked Muscular Dystrophy in Japan (CXMDJ).

Systemic Delivery of Allogenic Muscle Stem Cells Induces Long-Term Muscle Repair and Clinical Efficacy in Duchenne Muscular Dystrophy Dogs

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