2014Alzheimer s & DementiaRequires access

P1‐069: A PRESENILIN 1 MUTATION ALTERS APP LOCALIZATION IN HUMAN NEURONS

Grace Woodruff, Sol M. Reyna, Lawrence S.B. Goldstein

Open publisher page 0 citations

Abstract

Presenilin 1 (PS1) is the catalytic core of the γ-secretase complex that cleaves the Amyloid Precursor Protein (APP) and other type 1 transmembrane proteins. Mutations in PS1 are the most common cause of early onset Alzheimer's disease (AD). Despite extensive research into the mechanisms by which PS1 mutations cause AD, there is still uncertainty in the precise mechanisms by which PS1 mutations initiate disease. We utilized isogenic induced pluripotent stem cell (iPSC) derived neurons that harbor the PS1 Δe9 mutation to investigate neuronal phenotypes caused by mutant PS1. Additionally, we used quantitative immunofluorescence to measure APP localization and APP colocalization with endocytic markers. We report that the PS1 Δe9 mutation alters localization of APP such that there is decreased APP in axons and increased APP in the cell body. Additionally we found that there is decreased colocalization of APP with the early enodcytic marker Rab5 in axons. Our results suggest that abnormal APP localization may be an early event in progression of familial AD.

About this research paper

What this paper is about

Presenilin 1 (PS1) is the catalytic core of the γ-secretase complex that cleaves the Amyloid Precursor Protein (APP) and other type 1 transmembrane proteins. Mutations in PS1 are the most common cause of early onset Alzheimer's disease (AD). Despite extensive research into the mechanisms by which PS1 mutations cause AD, there is still uncertainty in the precise mechanisms by which PS1 mutations initiate disease. We utilized isogenic induced pluripotent stem cell (iPSC) derived neurons that harbor the PS1 Δe9 mutation to investigate neuronal phenotypes caused by mutant PS1. Additionally, we used quantitative immunofluorescence to measure APP localization and APP colocalization with endocytic markers. We report that the PS1 Δe9 mutation alters localization of APP such that there is decreased APP in axons and increased APP in the cell body. Additionally we found that there is decreased colocalization of APP with the early enodcytic marker Rab5 in axons. Our results suggest that abnormal APP localization may be an early event in progression of familial AD.

Why it matters

A significance statement is not available in the OpenAlex record.

Key contribution

A contribution statement is not available in the OpenAlex record.

Method / approach

Method details are not available in the OpenAlex metadata.

Main findings

Findings are not separately available in the OpenAlex metadata.

Limitations

Limitations are not available in the OpenAlex metadata.

Applications

Application details are not available in the OpenAlex metadata.

Available abstract

Presenilin 1 (PS1) is the catalytic core of the γ-secretase complex that cleaves the Amyloid Precursor Protein (APP) and other type 1 transmembrane proteins. Mutations in PS1 are the most common cause of early onset Alzheimer's disease (AD). Despite extensive research into the mechanisms by which PS1 mutations cause AD, there is still uncertainty in the precise mechanisms by which PS1 mutations initiate disease. We utilized isogenic induced pluripotent stem cell (iPSC) derived neurons that harbor the PS1 Δe9 mutation to investigate neuronal phenotypes caused by mutant PS1. Additionally, we used quantitative immunofluorescence to measure APP localization and APP colocalization with endocytic markers. We report that the PS1 Δe9 mutation alters localization of APP such that there is decreased APP in axons and increased APP in the cell body. Additionally we found that there is decreased colocalization of APP with the early enodcytic marker Rab5 in axons. Our results suggest that abnormal APP localization may be an early event in progression of familial AD.

Key concepts: Presenilin, Colocalization, Amyloid precursor protein, Biology, Mutation, Induced pluripotent stem cell, Phenotype, Mutant

Related papers

Back to paper searchBrowse research topicsOriginal source
P1‐069: A PRESENILIN 1 MUTATION ALTERS APP LOCALIZATION IN HUMAN NEURONS — Research Paper | ScholarLens