# Common themes of the biology of disease in polyglutamine disorders

> **NIH NIH R01** · WAYNE STATE UNIVERSITY · 2024 · $59,906

## Abstract

PROJECT SUMMARY
 The goals of this R01 Supplemental award are to: 1) find common and distinct themes of degeneration
 and neuroprotection in the family of polyglutamine (polyQ) disorders; and 2) enhance the professional
 and scientific training of Dr. Oluwademilae Nuga, postdoctoral fellow in the Todi laboratory at Wayne
State University School of Medicine.
The polyQ family comprises nine incurable diseases: Spinocerebellar Ataxias (SCAs) 1, 2, 3, 6, 7 and 17,
Huntington's disease (arguably the most famous member of the family), Dentatorubral-pallidoluysian atrophy
and Spinal and Bulbar Muscular Atrophy (also known as Kennedy's Disease). Much has been learned about
the biology of disease in the polyQ family, yet we lack therapeutic interventions in the clinic, indicating that we
still have much to learn about mechanisms underscoring them. Here, we propose to conduct unbiased and hy-
pothesis-targeted studies in mammalian cells and in Drosophila models of disease to uncover common themes
of disturbance at the cellular and animal level. The work that we propose seeks to enhance ongoing research
in the Todi lab, funded under R01NS086778 (2014-2028).
Specifically, the studies outlined for the two year timeframe of this proposal will use RNA-Seq and unbiased
proteomics from cells stably expressing full-length, wild type and disease-causing polyQ proteins to undertake
an unprecedented “birds-eye view” of shared and distinct mechanisms of degeneration. Unbiased studies will
then be conceptualized into targeted hypotheses to confirm and extend them in animal models of disease and
in patient-derived iNeuronal cells. The Todi lab recently completed the generation of a unique, unprecedented
family of isogenic Drosophila lines that express the wild-type or pathogenic version of each polyQ protein that
Dr. Nuga will use in this proposal.
Alongside the scientific work, Dr. Nuga will undergo thorough mentoring for further scientific and professional
growth, ensuring that by the end of this proposal she is very well positioned to apply for a K99/R00 award fo-
cused on her own data, which she can then build towards an independent career in academia.

## Key facts

- **NIH application ID:** 11081371
- **Project number:** 3R01NS086778-10A1S1
- **Recipient organization:** WAYNE STATE UNIVERSITY
- **Principal Investigator:** Sokol Todi
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $59,906
- **Award type:** 3
- **Project period:** 2024-07-01 → 2026-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/11081371

## Citation

> US National Institutes of Health, RePORTER application 11081371, Common themes of the biology of disease in polyglutamine disorders (3R01NS086778-10A1S1). Retrieved via AI Analytics 2026-05-26 from https://api.ai-analytics.org/grant/nih/11081371. Licensed CC0.

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