Middlebury
Browse

File(s) under embargo

Reason: Restricted to Campus

Characterizing the Role of GDE2 in the SOD1G93A Mouse Model ofAmyotrophic Lateral Sclerosis

thesis
posted on 2024-03-01, 02:05 authored by Thea Joseph

Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease that effects motor neurons (MNs) in the adult central nervous system (CNS). The disease’s etiology is poorly understood and there are limited treatments available. Uncovering the mechanisms of neurodegeneration involved in ALS could be crucial to developing more effective treatments. The glycerophosphodiester phosphodiesterase (GDE) family of enzymes are transmembrane proteins with the unique ability to cleave glycosylphosphatidylinositol (GPI) anchored proteins within their lipid tail. GDE2 (also known as GDPD5) is the best researched member of the of the GDE family. Much of what we know about the protein comes from research regarding its role in

neurodevelopment, but it is also shown to be neuroprotective in the adult mouse CNS. This paper expands on that research, showing that, while Gde2 is generally haplosufficient, in the presence of additional degenerative pressure from the SOD1G93A mutation designed to mimic human ALS, GDE2’s neuroprotective capacity towards spinal MNs is impaired. This suggests that GDE2 disfunction contributes to disease progression in ALS.

History

Institution

  • Middlebury College

Department or Program

  • Neuroscience

Academic Advisor

Clinton Cave, Ph.D.

Conditions

  • Restricted to Campus

Usage metrics

    Student Scholarship

    Categories

    Licence

    Exports

    RefWorks
    BibTeX
    Ref. manager
    Endnote
    DataCite
    NLM
    DC