All Conference Presentations, Performances and Exhibits
Investigation into Hungtington's Disease in a Mouse Stem Cell Model
Location
Guzman 114
Start Date
4-15-2016 3:00 PM
End Date
4-15-2016 3:30 PM
Student Type
Undergraduate - Honors
Faculty Mentor(s)
Kiowa Bower, Ph.D.
Presentation Format
Oral Presentation
Abstract/Description
Huntington's Disease (HD) is a progressive neurodegenerative disease with significant psychiatric and physical effects. This study utilizes two lines of neural stem cells (NSCs) as a cell culture model of HD. One cell line possesses 7 glutamine repeats (7Q) and the other expresses a mutant form of huntingtin (140Q) Preliminary evidence suggests that mHtt can promote gains of function in pathways that regulate cell motility and neurite outgrowth. This is consistent with neuropathological observations indicative of abnormal neurite growth in striatal spiny neurons in HD brain. In the first part of this study we characterized both the 7Q and 140Q cell lines and optimized a protocol for differentiation of these cells into mature neurons. With a working cell culture model of HD we then move on to elucidate the effect of mHTT expression on cellular phenotypes and protein expression. Preliminary studies and assay development were done in 7Q (wild-type) and 140Q (mutant Htt) knock-in, mouse neural stem cells and differentiated neurons. We used immunocytochemical labels to track proteins such as huntingtin and other relevant markers of neuronal differentiation. Examination of differentiated neural mouse stem cells in our model reveal definite induced changes in structural protein expression patterns in mHTT lines and enlargement of nuclear region.
Investigation into Hungtington's Disease in a Mouse Stem Cell Model
Guzman 114
Huntington's Disease (HD) is a progressive neurodegenerative disease with significant psychiatric and physical effects. This study utilizes two lines of neural stem cells (NSCs) as a cell culture model of HD. One cell line possesses 7 glutamine repeats (7Q) and the other expresses a mutant form of huntingtin (140Q) Preliminary evidence suggests that mHtt can promote gains of function in pathways that regulate cell motility and neurite outgrowth. This is consistent with neuropathological observations indicative of abnormal neurite growth in striatal spiny neurons in HD brain. In the first part of this study we characterized both the 7Q and 140Q cell lines and optimized a protocol for differentiation of these cells into mature neurons. With a working cell culture model of HD we then move on to elucidate the effect of mHTT expression on cellular phenotypes and protein expression. Preliminary studies and assay development were done in 7Q (wild-type) and 140Q (mutant Htt) knock-in, mouse neural stem cells and differentiated neurons. We used immunocytochemical labels to track proteins such as huntingtin and other relevant markers of neuronal differentiation. Examination of differentiated neural mouse stem cells in our model reveal definite induced changes in structural protein expression patterns in mHTT lines and enlargement of nuclear region.