MPD in Telomerase Null Mice
Abstract
The molecular mechanisms and genetic pathways involved in the pathogenesis of myeloproliferative disease (MPDs) are not well understood. Telomore maintenance and the cellular responses to telomere dysfunction have been proposed to play crucial roles in the processes of genomic stability, aging, organ homeostasis and tumorigenesis. Our recent observation linking telomere dysfunction to an MPF like phenotype with thrombocytosis in the telomerase mutant mouse model provides an unique genetic platform to explore the molecular mechanisms by which telomere dysfunction contributes to the pathogenesis of MPD and essential thrombocytosis (ET). We hypothesize that mice engineered to have critically short telomeres will develop genomic instability which predispose these mice to the development of MPD/ET. In vitro and in vivo analyses of the hematopoietic compartment from these mutant mouse cohorts as a function of age and generation in the last 2 years have elucidated some of the genetic pathways that are involved in the pathogenesis of MPDs/ET.
Document Details
- Document Type
- Technical Report
- Publication Date
- Sep 01, 2007
- Accession Number
- ADA587644
Entities
People
- Kwok-kin Wong
Organizations
- Dana–Farber Cancer Institute