Wolfram Syndrome, a rare genetic disorder, is caused in most cases by mutations in the WFS1 gene whose product negatively regulates ATF6-alpha, causing hyperactivation of that pathway, ER stress, and pancreatic beta-cell death. In this project, we tested various knockout and wild-type cell lines to distinguish which ER pathways are important to stress in embryonic fibroblasts. The results showed that ATF6-alpha, although important in adult cells, was less important in embryonic cells, while PERK and IRE1-alpha pathways are important embryonically

• This report represents the work of one or more WPI undergraduate students submitted to the faculty as evidence of completion of a degree requirement. WPI routinely publishes these reports on its website without editorial or peer review.

Creator

• Huston, Christopher Wilson
• Broberg, Jared Christopher
• Goodsell, Kyle

Contributors

• Fumihiko Urano

Publisher

• Worcester Polytechnic Institute

Identifier

• E-project-042212-165632

Advisor

• Adams, David S.

Year

• 2012

Sponsor

• UMASS Medical Center

Date created

• 2012-04-22

Resource type

• Major Qualifying Project

Major

• Biology & Biotechnology

Rights statement

• In Copyright