F Gaschen

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Absence of dystrophin, as found in Duchenne boys, mdx mice and HFMD cats, leads to destabilization of the sarcolemmal-associated protein complex. Gene and cell therapy strategies aim to restore the dystrophin-associated protein complex. In order to better understand the cellular events involved in such therapy in feline and human muscular dystrophy, we(More)
Dystrophin deficiency causes Duchenne muscular dystrophy (DMD). Hypertrophic feline muscular dystrophy (HFMD) is a homologous animal model of DMD. Our objective was to investigate the early changes caused by dystrophin deficiency in skeletal muscle of cats of 3-4 and 6-9 months. Obvious histological lesions were already present in the younger cats, and they(More)
Utrophin, an autosomal homologue of dystrophin, has been suggested as a possible therapeutic replacement of dystrophin in Duchenne or Becker muscular dystrophies (DMD/BMD). We have undertaken this study to examine the expression of utrophin in the skeletal muscle of dystrophin-deficient cats, a spontaneous animal model for dystrophinopathy. Dystrophin was(More)
Standard needle electromyography (EMG) of 56 muscles and nerve conduction velocities (NCV) of the ulnar and common peroneal nerves were investigated in each of six cats affected with hypertrophic feline muscular dystrophy, 10 related heterozygote carriers and 10 normal cats. The EMG findings were considered normal in carrier and control cats, and consisted(More)
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