Faculty Advisor

Adams, David S.

Center

UMMC / University of Massachusetts Medical Center

Abstract

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

Publisher

Worcester Polytechnic Institute

Date Accepted

April 2012

Major

Biology and Biotechnology

Project Type

Major Qualifying Project

Accessibility

Unrestricted

Advisor Department

Biology and Biotechnology

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