In our experimental conditions, although there was an increased proportion of misshapen nuclei (Supplemental Fig. S3B) in ADLD fibroblasts and increased LB1 levels (LB1 immunoreactive area/DNA stained area ratio, CTR 84.9±5.9; ADLD 185.0±10.8; P<0.01), we did not observe any gross abnormalities of the localization and levels of sc-35 (Fig. 7A, B), fibrillarin (Fig. 7C, D), LAP2β (Fig. 7E-F), trimethyl histone H3 (Fig. 7G, H), and nucleoporins (Fig. 7I, J). The gene discussed is FBL; the disease is adult-onset autosomal dominant demyelinating leukodystrophy.