Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model.

  title={Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model.},
  author={Mary Y Heng and Shu-Ting Lin and Laure Verret and Yong Huang and Sherry Kamiya and Quasar Salem Padiath and Ying Tong and Jorge J. Palop and Eric H. Huang and Louis J. Pt{\'a}cek and Ying-Hui Fu},
  journal={The Journal of clinical investigation},
  volume={123 6},
Adult-onset autosomal-dominant leukodystrophy (ADLD) is a progressive and fatal neurological disorder characterized by early autonomic dysfunction, cognitive impairment, pyramidal tract and cerebellar dysfunction, and white matter loss in the central nervous system. ADLD is caused by duplication of the LMNB1 gene, which results in increased lamin B1 transcripts and protein expression. How duplication of LMNB1 leads to myelin defects is unknown. To address this question, we developed a mouse… CONTINUE READING


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