Review Article

Untethering the Nuclear Envelope and Cytoskeleton: Biologically Distinct Dystonias Arising from a Common Cellular Dysfunction

Figure 2

Structural representation of dystonin protein isoforms. (a) Illustration of the modular domain structure of tissue specific dystonin isoforms. Three tissue-specific isoforms have been identified; muscle (dystonin-b), neuronal (dystonin-a), and epithelial (dystonin-e). (b) Dystonin-a and b isoforms vary at the N-terminus, resulting in three distinct isoforms. The isoform 1 variant possesses a sequence coding for an ABD at the N-terminus. The isoform 2 variant contains sequence coding for a highly conserved N-terminal TM domain, while the isoform 3 variant contains sequence coding for a conserved MYR motif.
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(a)
634214.fig.002b
(b)