A lamin A/C variant causing striated muscle disease provides insights into filament organization
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ABSTRACT:
SUBMITTER: Ohad Medalia
PROVIDER: EMPIAR-10601 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
A lamin A/C variant causing striated muscle disease provides insights into filament organization.
Kronenberg-Tenga Rafael R Tatli Meltem M Eibauer Matthias M Wu Wei W Shin Ji-Yeon JY Bonne Gisèle G Worman Howard J HJ Medalia Ohad O
Journal of cell science 20210322 6
The <i>LMNA</i> gene encodes the A-type lamins, which polymerize into ∼3.5-nm-thick filaments and, together with B-type lamins and associated proteins, form the nuclear lamina. Mutations in <i>LMNA</i> cause a wide variety of pathologies. In this study, we analyzed the nuclear lamina of embryonic fibroblasts from <i>Lmna</i><sup>H222P/H222P</sup> mice, which develop cardiomyopathy and muscular dystrophy. Although the organization of the lamina appeared unaltered, there were changes in chromatin ...[more]
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