Ontology highlight
ABSTRACT:
SUBMITTER: Chen X
PROVIDER: S-EPMC7853716 | biostudies-literature | 2021 Feb
REPOSITORIES: biostudies-literature
Chen Xue X Yao Haidong H Kashif Muhammad M Revêchon Gwladys G Eriksson Maria M Hu Jianjiang J Wang Ting T Liu Yiran Y Tüksammel Elin E Strömblad Staffan S Ahearn Ian M IM Philips Mark R MR Wiel Clotilde C Ibrahim Mohamed X MX Bergo Martin O MO
eLife 20210202
A farnesylated and methylated form of prelamin A called progerin causes Hutchinson-Gilford progeria syndrome (HGPS). Inhibiting progerin methylation by inactivating the isoprenylcysteine carboxylmethyltransferase (ICMT) gene stimulates proliferation of HGPS cells and improves survival of <i>Zmpste24</i>-deficient mice. However, we don't know whether <i>Icmt</i> inactivation improves phenotypes in an authentic HGPS mouse model. Moreover, it is unknown whether pharmacologic targeting of ICMT would ...[more]