# Dietary Contribution in Cerebellar Ataxia

> **NIH NIH R03** · COLUMBIA UNIVERSITY HEALTH SCIENCES · 2020 · $81,000

## Abstract

PROJECT SUMMARY/ ABSTRACT
Cerebellar ataxia is a disabling symptom and can have a variety of causes, among which
immune-mediated gluten ataxia is one of the most common causes. Gluten ataxia is associated
with autoimmunity against transglutaminase 6 (TG6) and some patients with gluten ataxia will
respond to gluten-free diet. However, the responses to gluten-free diet in patients with gluten
ataxia might be variable and there is no clear understanding how TG6 could play a role in ataxia
and/or in response to gluten-free diet. Recently, genetic mutations of TGM6, the gene that
encodes TG6 protein, have been identified to cause spinocerebellar ataxia type 35 (SCA35).
We recently identified a SCA35 patient with a truncated TGM6 mutation and cerebellar ataxia,
and his ataxia improved with gluten-free diet, demonstrating that both genetic and immune-
mediated causes can converge at the TG6 biology, which serves as a window to probe the
dietary contribution in neurological disorders. In this R03 proposal, we will establish the first
mouse model of SCA35 and monitor its responses to gluten-diet in ataxia progression (Specific
Aim 1), neurophysiology (Specific Aim 2), and neuropathology (Specific Aim 3). This mouse
model will serve as a benchmark for future mechanistic studies to understand gluten biology
and cerebellar ataxia.

## Key facts

- **NIH application ID:** 10017360
- **Project number:** 5R03NS114871-02
- **Recipient organization:** COLUMBIA UNIVERSITY HEALTH SCIENCES
- **Principal Investigator:** Sheng-Han Kuo
- **Activity code:** R03 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $81,000
- **Award type:** 5
- **Project period:** 2019-09-15 → 2021-08-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10017360

## Citation

> US National Institutes of Health, RePORTER application 10017360, Dietary Contribution in Cerebellar Ataxia (5R03NS114871-02). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10017360. Licensed CC0.

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