Iovane, Valentina (2011) A SPONTANEOUS MOUSE MODEL OF X-LINKED MYOPATHY WITH EXCESSIVE AUTOPHAGY. [Tesi di dottorato] (Inedito)

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Tipologia del documento: Tesi di dottorato
Lingua: English
Titolo: A SPONTANEOUS MOUSE MODEL OF X-LINKED MYOPATHY WITH EXCESSIVE AUTOPHAGY
Autori:
AutoreEmail
Iovane, Valentinavale.iovane@gmail.com
Data: 30 Novembre 2011
Numero di pagine: 57
Istituzione: Università degli Studi di Napoli Federico II
Dipartimento: Strutture, funzioni e tecnologie biologiche
Scuola di dottorato: Scienze veterinarie per la produzione e la sanità
Dottorato: Organismi modello nella ricerca biomedica e veterinaria
Ciclo di dottorato: 24
Coordinatore del Corso di dottorato:
nomeemail
De Girolamo, Paolodegirola@unina.it
Tutor:
nomeemail
Papparella, Serenellapapparel@unina.it
Paciello, Orlandopaciello@unina.it
Data: 30 Novembre 2011
Numero di pagine: 57
Parole chiave: X-Linked Vacuolar myopathy with Excessive Autophagy, Autophagy, muscle, mouse model
Settori scientifico-disciplinari del MIUR: Area 07 - Scienze agrarie e veterinarie > VET/03 - Patologia generale e anatomia patologica veterinaria
Depositato il: 09 Dic 2011 11:11
Ultima modifica: 30 Apr 2014 19:48
URI: http://www.fedoa.unina.it/id/eprint/8824
DOI: 10.6092/UNINA/FEDOA/8824

Abstract

the subject of the thesis research project, aims to characterize a murine model for spontaneous muscle pathology comparable to human "X-Linked Vacuolar myopathy with Excessive Autophagy (XMEA)". Along with Danon disease, these myopathies are characterized by the accumulation of vacuoles within the myofiber. In 1988, Kalimo et al. have described five cases (all males) who had progressive proximal myopathy, which did not involve the heart muscle. The muscle biopsies of all patients showed numerous vacuoles, sarcoplasmic and subsarcolemmal, many of which were positive for the lysosomal enzyme, for the morphological appearance described, was proposed the term: X-Linked Vacuolar myopathy with Excessive Autophagy (XMEA). We used crosses of strain C57/BL6 mice of different ages and sex, we processed triceps muscles, quadriceps femoris and the cranial tibial muscle and made morphological, histochemical and immunohistochemical staining on this samples. From laboratory tests performed on muscle biopsies, we found the presence of numerous vacuoles within muscle fibers, only in males. All these characters are comparable to XMEA. The availability of a well characterized mouse model it may help define the etiopathogenesis of this disease.

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