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Extra resources for Anemia - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References
The subaims of this section are to generate a colony of FANCG (-/-, -/+, and +/+) mice, to characterize the hematopoietic system in the FANCG knock-out mouse, to characterize the stem cell population in FANCG knock-out, and to perform competitive repopulation studies, comparing the relative engraftment of FANCG (-/-) cells versus FANCG (+/+) cells. In specific aim 2, we will further develop murine models for Fanconi Anemia Gene Therapy in order to compare the transduction of bone marrow cells from FANCC, FANCA, and FANCG knockout mouse models with either pMMP (murine) retroviral vectors or lentiviral vectors.
It is possible that alphaSpllsigma* acts as a scaffold to help align or enhance interaction between proteins involved in the repair of interstrand cross-links and proteins that interact with FAA and FAC. The present proposal will address this by first determining the isoform of the alphaSpllsigma* we have identified and producing a recombinant protein that can be used in further studies. Exactly what proteins are associated with the FAA-FACalphaSpllsigma* complex, whether any of these proteins have binding affinity for DNA containing interstrand cross-links, and whether there is a deficiency in any of these proteins in FA-A and FA-C cells will be determined.
This aim will be closely aligned with our new Fanconi Anemia Diagnostic and Clinical Center at the DFCI, which has provided us with the opportunity to collect and subtype cell lines from many FA patients. We will compare the ionizing radiation (IR) sensitivity and bleomycin sensitivity of all FA complementation groups, and will examine these cell lines for defects in Non-Homologous End joining (NHEJ) and Homologous Recombination (HR). Generate_Screen 22 Anemia · Project Title: FANCONI ANEMIA GENE PATHWAY IN RADIATION RESPONSES Principal Investigator & Institution: Thompson, Lawrence H.