Data di Pubblicazione:
2009
Citazione:
Muscular Dystrophy with Reduced β-Sarcoglycan in a Cat / C. Salvadori, G. Vattemi, R. Lombardo, M. Marini, C. Cantile, G.D. Shelton. - In: JOURNAL OF COMPARATIVE PATHOLOGY. - ISSN 0021-9975. - 140:4(2009), pp. 278-282. [10.1016/j.jcpa.2008.12.003]
Abstract:
A partial β-sarcoglycan (SG) deficiency with retention of other components of the SG complex (SGC) is described in 6-month-old, intact male domestic shorthaired kitten that was referred for evaluation of weakness, reluctance to move and dyspnoea. Neurological deficits were restricted to the neuromuscular system. Muscle biopsy revealed moderate variability in myofibre size, with numerous atrophic rounded fibres, rare myofibre necrosis, regeneration and moderate perimysial and endomysial fibrosis. Immunohistochemistry revealed decreased expression of β- and γ-SG and western blotting revealed markedly decreased β-SG with normal expression of α-, γ- and δ-SG, caveolin-3 and calpain-3. Sarcoglycanopathy has not previously been described in cats. In human and canine sarcoglycanopathies the deficiency in any one of the SGs leads to secondary deficiency of the entire SGC. Such spontaneously arising muscular disease in animals can provide valuable models for equivalent human disorders.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
animal model; cat; muscular dystrophy; sarcoglycan deficiency
Elenco autori:
C. Salvadori, G. Vattemi, R. Lombardo, M. Marini, C. Cantile, G.D. Shelton
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