Publications by authors named "Ester Neufeld"
Article Synopsis
- Mutations in the LMNA gene or lack of ZMPSTE26 activity lead to faulty lamin A processing and contribute to early aging diseases like Hutchinson Gilford progeria syndrome (HGPS).
- Fibroblasts from HGPS show age-related changes in nuclear structure that can be reversed using farnesyl transferase inhibitors (FTIs), which also extend the lifespan of mice models with HGPS.
- In adult C. elegans, reducing lamin expression mimics normal aging changes in nuclear architecture, and while FTIs improve motility in aging worms, they do not significantly increase lifespan.
Age-related changes of nuclear architecture in Caenorhabditis elegans.
Proc Natl Acad Sci U S A
November 2005
Mutations in lamins cause premature aging syndromes in humans, including the Hutchinson-Gilford Progeria Syndrome (HGPS) and Atypical Werner Syndrome. It has been shown that HGPS cells in culture undergo age-dependent progressive changes in nuclear architecture. However, it is unknown whether similar changes in nuclear architecture occur during the normal aging process.
View Article and Find Full Text PDFEmerin and MAN1 are LEM domain-containing integral membrane proteins of the vertebrate nuclear envelope. The function of MAN1 is unknown, whereas emerin is known to interact with nuclear lamins, barrier-to-autointegration factor (BAF), nesprin-1 alpha, and a transcription repressor. Mutations in emerin cause X-linked recessive Emery-Dreifuss muscular dystrophy.
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