Journal article
Primary over-expression of AβPP in muscle does not lead to the development of inclusion body myositis in a new lineage of the MCK-AβPP transgenic mouse
International Journal of Experimental Pathology, Vol.94(6), pp.418-425
2013
Abstract
The aim of this study is to determine whether primary over-expression of AβPP in skeletal muscle results in the development of features of inclusion body myositis (IBM) in a new lineage of the MCK-AβPP transgenic mouse. Quantitative histological, immunohistochemical and western blotting studies were performed on muscles from 3 to 18 month old transgenic and wild-type C57BL6/SJL mice. Electron microscopy was also performed on muscle sections from selected animals. Although western blotting confirmed that there was over-expression of full length AβPP in transgenic mouse muscles, deposition of amyloid-β and fibrillar amyloid could not be demonstrated histochemically or with electron microscopy. Additionally, other changes typical of IBM such as rimmed vacuoles, cytochrome C oxidase-deficient fibres, upregulation of MHC antigens, lymphocytic inflammatory infiltration and T cell fibre invasion were absent. The most prominent finding in both transgenic and wild-type animals was the presence of tubular aggregates which was age-related and largely restricted to male animals. Expression of full length AβPP in this MCK-AβPP mouse lineage did not reach the levels required for immunodetection or deposition of amyloid-β as in the original transgenic strains, and was not associated with the development of pathological features of IBM. These negative results emphasise the potential pitfalls of re-deriving transgenic mouse strains in different laboratories.
Details
- Title
- Primary over-expression of AβPP in muscle does not lead to the development of inclusion body myositis in a new lineage of the MCK-AβPP transgenic mouse
- Authors/Creators
- Y-B Luo (Author/Creator) - Perron Institute for Neurological and Translational ScienceR.D. Johnsen (Author/Creator) - Perron Institute for Neurological and Translational ScienceL. Griffiths (Author/Creator) - Royal Perth HospitalM. Needham (Author/Creator) - Perron Institute for Neurological and Translational ScienceV.A. Fabian (Author/Creator) - Royal Perth HospitalS. Fletcher (Author/Creator) - Perron Institute for Neurological and Translational ScienceS.D. Wilton (Author/Creator) - Perron Institute for Neurological and Translational ScienceF.L. Mastaglia (Author/Creator) - Perron Institute for Neurological and Translational Science
- Publication Details
- International Journal of Experimental Pathology, Vol.94(6), pp.418-425
- Publisher
- Wiley
- Identifiers
- 991005541391507891
- Copyright
- © 2013 The Authors
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
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- Citation topics
- 1 Clinical & Life Sciences
- 1.52 Neurodegenerative Diseases
- 1.52.57 Alzheimer's Mechanisms
- Web Of Science research areas
- Pathology
- ESI research areas
- Clinical Medicine