Mice overexpressing lamin B1 in oligodendrocytes recapitulate the age-dependent motor signs, but not the early autonomic cardiovascular dysfunction of autosomal-dominant leukodystrophy (ADLD).

doi:10.1016/j.expneurol.2017.12.006

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Article

Mice overexpressing lamin B1 in oligodendrocytes recapitulate the age-dependent motor signs, but not the early autonomic cardiovascular dysfunction of autosomal-dominant leukodystrophy (ADLD).

Experimental neurology - 1 Mar 2018

Lo Martire Viviana, Alvente Sara, Bastianini Stefano, Berteotti Chiara, Bombardi Cristiano, Calandra-Buonaura Giovanna, Capellari Sabina, Cohen Gary, Cortelli Pietro, Gasparini Laura, Padiath Quasar, Valli Alice, Zoccoli Giovanna, Silvani Alessandro

Abstract excerpt

Autosomal dominant leukodystrophy (ADLD) is a rare adult-onset demyelinating disease caused by overexpression of lamin B1, a nuclear lamina filament. Early autonomic dysfunction involving the cardiovascular system before progressive somatic motor dysfunction is a striking feature of most cases of ADLD. In the Plp-FLAG-LMNB1 transgenic mouse model, lamin B1 overexpression in oligodendrocytes elicits somatic motor...

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PMID: 29262292
DOI: 10.1016/j.expneurol.2017.12.006
PMCID: PMC5809293

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LMNB1

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