Oct-1 recruitment to the nuclear envelope in adult-onset autosomal dominant leukodystrophy

► Lamin B1 is accumulated in the nuclear lamina of skin and muscle cells in ADLD. ► Lamin B1 accumulation in ADLD is associated with Oct-1 recruitment. ► Lamin B1 and Oct-1 amount in nuclei are affected by protein degradation rate. ► Skeletal muscle tissue abnormalities are involved in ADLD. ► The severity of ADLD phenotype is related to the amount of accumulated lamin B1.