Abstract:
INTRODUCTION:We adopted a proteomics-based approach to gain insights into phenotypic differences between A/J and B10.SJL murine dysferlinopathy models. METHODS:We optimized immunoblotting of dysferlin by preparing homogenates of the tibialis anterior (TA) muscle under several different conditions. We compared TA muscles of control, A/J, and B10.SJL mice for levels of dysferlin; dysferlin's partners MG53, annexin-A2, and caveolin-3; and the endoplasmic reticulum (ER) stress marker CHOP. We performed immunoelectron microscopy on control rat TA muscle to determine the precise location of dysferlin. RESULTS:RIPA (radioimmunoprecipitation assay) buffer and sonication improves immunoblotting of dysferlin. The ER stress marker CHOP is elevated in A/J muscle. Dysferlin is localized mostly to membranes close to the Z-disk that have been reported to be part of the Golgi, ER, and sarcoplasmic reticulum (SR) networks. CONCLUSIONS:ER stress might underlie phenotypic differences between A/J and B10.SJL mice and play a role in human dysferlinopathies.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Mueller AL,Desmond PF,Hsia RC,Roche JAdoi
10.1002/mus.24220subject
Has Abstractpub_date
2014-08-01 00:00:00pages
286-9issue
2eissn
0148-639Xissn
1097-4598journal_volume
50pub_type
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