# Ciliary Hedgehog signaling during adult tissue repair and disease

> **NIH NIH R01** · UNIVERSITY OF FLORIDA · 2024 · $341,281

## Abstract

Project Summary
Fatty fibrosis, the replacement of healthy muscle tissue with fat and fibrotic scar tissue, is a prominent feature of
chronic muscle diseases such as Duchenne muscular dystrophy (DMD), sarcopenia, the age-related loss of
skeletal muscle and strength, in addition to obesity, and diabetes. This project will identify and characterize the
cellular and molecular mechanisms by which intramuscular fat forms, and the functional consequences of
intramuscular fat on muscle health. Recent evidence points to ciliary Hedgehog signaling as a potent ant-
adipogenic signal during muscle regeneration and in mdx mice, a mouse model of DMD. In addition, Hedgehog
promotes muscle repair and prevents the decline in myofiber size in mdx mice. By what mechanism(s) Hedgehog
acts to balance fat formation and muscle regeneration is unknown. This proposal will use innovative and powerful
mouse models to identify the role of Hedgehog signaling during muscle regeneration in the following aims: 1)
Identify and characterize the responsible Hedgehog ligand; 2) determine which cell types respond to Hedgehog
signaling; and 3) demonstrate if intramuscular fat directly affects muscle health. Taken together, this proposal
will provide insights into the origin and function of intramuscular fat and will aid in the search for novel therapeutic
interventions into human diseases affected by fatty fibrosis.

## Key facts

- **NIH application ID:** 10817032
- **Project number:** 5R01AR079449-03
- **Recipient organization:** UNIVERSITY OF FLORIDA
- **Principal Investigator:** Daniel Kopinke
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $341,281
- **Award type:** 5
- **Project period:** 2022-04-01 → 2027-03-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10817032

## Citation

> US National Institutes of Health, RePORTER application 10817032, Ciliary Hedgehog signaling during adult tissue repair and disease (5R01AR079449-03). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/10817032. Licensed CC0.

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