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Article

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

The Journal of clinical investigation - 1 Jun 2013

Heng Mary Y, Lin Shu-Ting, Verret Laure, Huang Yong, Kamiya Sherry, Padiath Quasar S, Tong Ying, Palop Jorge J, Huang Eric J, Ptáček Louis J, Fu Ying-Hui

Abstract excerpt

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...

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Source

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Identifiers

PMID: 23676464
DOI: 10.1172/JCI66737
PMCID: PMC3668844

Genes

LMNB1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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