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Tibbetts Lab

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Research focus

1. Research projects in our lab broadly pertain to genomic surveillance and molecular mechanisms of neurodegeneration in ALS.

2. Areas of focus:

(i) Genome protection. Our laboratory is interested in mechanisms of genome surveillance and tumor suppression. Much of our work is focused on the regulatory relationships between the ATM protein kinase and CREB/ATF family transcription factors (TFs) and the broader roles of CREB/ATF family transcription factors in genome protection and DNA repair. We are addressing the functional significance of this pathway using cell culture models and gene-targeted mice. A related project is focusing on an emergent role for RNA-bindings proteins (RBPs) in DNA repair. The long-term goal of these studies is to understand fundamental aspects of DNA damage repair and response that can be used to guide radio- and chemotherapeutic strategies for cancer patients.

(ii) Molecular cell biology of ALS-associated proteins. ALS is a fatal neurodegenerative disease that destroys motor neurons. Recent genetic advances have identified a handful of genes that are critically involved in the disease process, including the RNA-binding proteins TDP-43 and FUS/TLS, the proteasome targeting factor UBQLN2, and the uncharacterized open reading frame, C9ORF72. We are using cell culture, Drosophila, and mouse models to understand how ALS-associated mutations impact the function, stability, and regulation of these proteins.