Figure 5.

Responsivity of individual splice events and overall splicing index (mDSI) to allelic inactivation of Mbnl1/Mbnl2 or increments of CUG^exp expression. (A) Based on targeted splice sequencing, mDSI was determined in quadriceps for 97 mice having the indicated genotypes. Note that CUG^exp tracts in HSAXLRe mice are twice as long as in HSALR mice. (B) PSI responsivity of individual splice events plotted against mDSI, a proxy for residual Mbnl activity. Some events, such as Neb or Phka1, are more responsive to moderate reduction of Mbnl, whereas others, such as Clcn1, are more responsive to changes when CUG^exp levels are quite high or Mbnl levels low.