The BAF A12T mutation disrupts lamin A/C interaction, impairing robust repair of nuclear envelope ruptures in Nestor–Guillermo progeria syndrome cells

Abstract Nestor–Guillermo progeria syndrome (NGPS) is caused by a homozygous alanine-to-threonine mutation at position 12 (A12T) in barrier-to-autointegration factor (BAF). It is characterized by accelerated aging with severe skeletal abnormalities. BAF is an essential protein binding to DNA and nuc...

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Veröffentlicht in:Nucleic acids research 2022-09, Vol.50 (16), p.9260-9278
Hauptverfasser: Janssen, Anne, Marcelot, Agathe, Breusegem, Sophia, Legrand, Pierre, Zinn-Justin, Sophie, Larrieu, Delphine
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Sprache:eng
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