Lin, Haihuan and Mensch, Juliane and Haschke, Maria and Jager, Kathrin and Kottgen, Brigitte and Dernedde, Jens and Orso, Evelyn and Walter, Michael (2022) Establishment and Characterization of hTERT Immortalized Hutchinson-Gilford Progeria Fibroblast Cell Lines. CELLS, 11 (18): 2784. ISSN , 2073-4409
Full text not available from this repository. (Request a copy)Abstract
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging syndrome caused by a dominant mutation in the LMNA gene. Previous research has shown that the ectopic expression of the catalytic subunit of telomerase (hTERT) can elongate the telomeres of the patients' fibroblasts. Here, we established five immortalized HGP fibroblast cell lines using retroviral infection with the catalytic subunit of hTERT. Immortalization enhanced the proliferative life span by at least 50 population doublings (PDs). The number of cells with typical senescence signs was reduced by 63 + 17%. Furthermore, the growth increase and phenotype improvement occurred with a lag phase of 50-100 days and was not dependent on the degree of telomere elongation. The initial telomeric stabilization after hTERT infection and relatively low amounts of hTERT mRNA were sufficient for the phenotype improvement but the retroviral infection procedure was associated with transient cell stress. Our data have implications for therapeutic strategies in HGP and other premature aging syndromes.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | TELOMERE LENGTH; LAMIN; SENESCENCE; NUCLEAR; DYSFUNCTION; MUTATIONS; PHENOTYPE; Hutchinson-Gilford progeria syndrome (HGPS); telomerase; telomere length; cell senescence; beta-galactosidase |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 01 Feb 2024 15:11 |
| Last Modified: | 01 Feb 2024 15:11 |
| URI: | https://pred.uni-regensburg.de/id/eprint/58336 |
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