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Intermediate filament (IF) proteins constitute a unique part of the cytoskeleton and are present in high abundance in tissues such as skin, muscle, and the central and peripheral nervous systems. In general, their properties suggest that IFs play important roles in maintaining the mechanical stability of tissues undergoing severe mechanical stress. However, the lack of cytoplasmic IFs in the nervous...
Bpag1/dystonin proteins are giant cytoskeletal interacting proteins postulated to cross-link cytoskeletal filaments and thereby maintain cellular integrity. Loss of function of non-epithelial Bpag1 isoforms results in neuromuscular dysfunction and early postnatal death in mice. Multiple Bpag1 transcripts have been described, including those encoding protein isoforms that vary at the N-terminal end...
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