# Novel chaperones and neurodegeneration

> **NIH NIH R01** · DUKE UNIVERSITY · 2020 · $350,553

## Abstract

Abstract
The polyglutamine (polyQ) diseases are a class of nine neurodegenerative diseases caused by the expansion
of a polyQ tract in specific proteins. Expansion of the polyQ tract results in protein aggregation and
neurodegeneration. Currently there are no curative treatments for the polyQ diseases. One potential therapy for
the polyQ diseases is the development of strategies to reduce polyQ aggregation. Interestingly we and others
have found that one organism, Dictyostelium discoideum, is naturally resist to polyQ aggregation. Further work
from our laboratory has identified a novel molecular chaperone we named serine rich chaperone protein 1
(SRCP1) that is both necessary for Dictyostelium’s resistance to polyQ aggregation and sufficient to impart
resistance to polyQ aggregation to other organisms. In this application we propose to 1) determine the molecular
mechanism SRCP1 utilizes to recognize polyQ expanded proteins and suppress their aggregation; 2) determine
if SRCP1 can suppress protein aggregation and behavioral deficits in a mouse model of Huntington’s disease;
and 3) determine if SRCP1 is capable of suppressing polyQ aggregation in a mouse model of Spinocerebellar
ataxia type 3. Together these studies will determine the mechanism SRCP1 utilizes to suppress polyQ
aggregation and determine if SRCP1 has the ability to prevent or reverse disease phenotypes in mouse models
of polyQ disease.
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## Key facts

- **NIH application ID:** 9957136
- **Project number:** 5R01NS112191-02
- **Recipient organization:** DUKE UNIVERSITY
- **Principal Investigator:** Kenneth Matthew Scaglione
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $350,553
- **Award type:** 5
- **Project period:** 2019-07-01 → 2024-04-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9957136

## Citation

> US National Institutes of Health, RePORTER application 9957136, Novel chaperones and neurodegeneration (5R01NS112191-02). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/9957136. Licensed CC0.

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