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: | Autore Email Iovane, Valentina vale.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: | nome email De Girolamo, Paolo degirola@unina.it |
| Tutor: | nome email Papparella, Serenella papparel@unina.it Paciello, Orlando paciello@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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