Disrupted structure and aberrant function of CHIP mediates the loss of motor and cognitive function in preclinical models of SCAR16
Immuno-stained sagittal sections of either a wild-type mouse cerebellum (left) or a cerebellum isolated from a mouse engineered with the T246M mutation in CHIP (right). The T246M mutation results in a robust decrease in CHIP expression (white) in the Purkinje cells, ultimately leading to the degeneration of Purkinje cells and a loss in motor function, mimicking the human disease known as SCAR16. In red is a false coloring of the methyl blue counterstain, indicating the molecular layer of the cerebellum.