Tauopathies have been conventionally classified from a pathological perspective into two groups—(A) Primary tauopathies where tau is the predominant pathology including three repeat (3R-) and four repeat (4R-) tauopathies, (B) Secondary tauopathies where additional etiologies (e.g., amyloid, trauma, and autoimmune) are involved for tau deposition (Figure 1) (8). This evidence concerns the gene MAPT and tauopathy.