Biochemical data on CUGBP In vivo
|
|
|
|
Coimmunoprecipitation with mut RNA |
Yes |
|
Ab to CUGBP recovers mut RNA |
HMW complexes by SEC |
Yes |
|
All CUGBP in mut RNA complex |
Increased effect with longer repeat |
Yes |
|
Prolonged CUGBP half-life, accumulation, increased splicing abnormalities |
Increased CUGBP (pharmacodynamics) |
Yes |
|
Level as predicted by mut RNA binding |
Increased effect with high levels of short repeat |
Yes |
|
CUGBP accumulation, splice abnormalities, phenotypic DM |
In vitro |
|
|
|
Increased binding with longer repeat |
Yes |
No |
For yes, max n=123; RNA-free |
|
|
|
For no, max n=90; RNA-blocked |
|
|
|
|
Morphological data on CUGBP |
In vitro
|
|
|
|
Binding to mut RNA |
Yes |
|
By EM, binds to ss CUGn regions |
In vivo
|
|
|
|
Colocalization with mut RNA foci |
|
No |
Diffuse nuclear distribution for CUGBP |