VCP mediated mutagenesis in Danio rerio to model motor neuron degeneration in ALS

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Jones, Stephen
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Symposium on Undergraduate Research and Creative Expression
Iowa State University Conferences and Symposia

The Symposium provides undergraduates from all academic disciplines with an opportunity to share their research with the university community and other guests through conference-style oral presentations. The Symposium represents part of a larger effort of Iowa State University to enhance, support, and celebrate undergraduate research activity.

Though coordinated by the University Honors Program, all undergraduate students are eligible and encouraged to participate in the Symposium. Undergraduates conducting research but not yet ready to present their work are encouraged to attend the Symposium to learn about the presentation process and students not currently involved in research are encouraged to attend the Symposium to learn about the broad range of undergraduate research activities that are taking place at ISU.

The first Symposium was held in April 2007. The 39 students who presented research and their mentors collectively represented all of ISU's Colleges: Agriculture and Life Sciences, Business, Design, Engineering, Human Sciences, Liberal Arts and Sciences, Veterinary Medicine, and the Graduate College. The event has grown to regularly include more than 100 students presenting on topics that span the broad range of disciplines studied at ISU.

Genetics, Development and Cell Biology

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the death of motor neurons. Once these nerve cells die, the patient’s muscles waste away, resulting in paralysis and eventually death. Two primary forms of ALS exist: Familial and Sporadic. Familial constitutes 5-10% of cases and is defined by ALS being present in one or more cases in a family’s lineage. Sporadic makes up 90-95% of ALS cases and is essentially when no family history exists with ALS but an individual has ALS. Mutations in SOD1 have been the most studied in regards to ALS. However there are many other genes linked to ALS that have not been studied. VCP is a gene that has been linked to several different diseases including familial versions of ALS. The protein has been linked to many different cellular processes including protein degradation and programmed cell death. To gain a better understanding into the development and eventual death of motor neurons, we are using both TAL-effector nuclease (TALEN) mediated mutagenesis and a VCP CRSPR to create zebrafish that are mutant for VCP. These mutant fish will hopefully allow us to create a new model of motor neuron degeneration or ALS.