Dominican University of California
 

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Presentation or Panel Title

Investigation into Hungtington's Disease in a Mouse Stem Cell Model

Presenter Information

Matthew Erbst, StudentFollow

Location

Guzman 114

Start Date

4-15-2016 3:00 PM

End Date

4-15-2016 3:30 PM

Department

Natural Sciences and Mathematics

Student Type

Undergraduate - Honors

Faculty Mentor

Kiowa Bower

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.

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Apr 15th, 3:00 PM Apr 15th, 3:30 PM

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.