Iovane, Valentina (2011) A SPONTANEOUS MOUSE MODEL OF X-LINKED MYOPATHY WITH EXCESSIVE AUTOPHAGY. [Tesi di dottorato] (Unpublished)
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| Item Type: | Tesi di dottorato |
|---|---|
| Resource language: | English |
| Title: | A SPONTANEOUS MOUSE MODEL OF X-LINKED MYOPATHY WITH EXCESSIVE AUTOPHAGY |
| Creators: | Creators Email Iovane, Valentina vale.iovane@gmail.com |
| Date: | 30 November 2011 |
| Number of Pages: | 57 |
| Institution: | Università degli Studi di Napoli Federico II |
| Department: | 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 |
| Date: | 30 November 2011 |
| Number of Pages: | 57 |
| Keywords: | 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 |
| Date Deposited: | 09 Dec 2011 11:11 |
| Last Modified: | 30 Apr 2014 19:48 |
| URI: | http://www.fedoa.unina.it/id/eprint/8824 |
| DOI: | 10.6092/UNINA/FEDOA/8824 |
Collection description
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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