# Development of proteomic-based ECM signatures for lung fibrosis

> **NIH NIH R21** · MEDICAL UNIVERSITY OF SOUTH CAROLINA · 2021 · $364,870

## Abstract

ABSTRACT
Systemic sclerosis (SSc; scleroderma) is an idiopathic disorder of connective tissue characterized by
increased production and deposition of collagen in the skin and internal organs such as the lungs. The
etiology of SSc is unknown, although the role of genetic influences, environmental insults and abnormal
immune function are subjects of active investigation. Interstitial lung disease (ILD) is a complication of SSc
and is currently the leading cause of death in patients with this disease. Idiopathic pulmonary fibrosis (IPF) is
also a fibrotic disease with high morbidity and mortality. We have a unique resource consisting of lung tissues
from normal donors, patients with SSc and patients with IPF as well as matching primary lung fibroblasts. We
have used the lung tissues to generate tissue microarrays (TMAs) that include normal lung, SSc lung, and
IPF lung cores on the same TMA. We hypothesize that alteration of the collagen proteomes, including post-
translational modifications, represents a novel and clinically relevant signature of pulmonary complications of
SSc and IPF. We further hypothesize that very specific sites of collagen hydroxylation regulate the response
of primary adult human lung fibroblasts. We propose to use a novel collagen-targeting proteomic approach
to localize and measure collagen types and post- translational modifications within the lung tissues and
identify disease (SSc vs. IPF) and phenotype (normal vs. fibrosis) changes. Identified post-translationally
modified collagen peptides will be synthesized to test the response of fibroblasts from normal, SSc and IPF
lung tissues to the peptides. Our findings will facilitate the development of targeted therapies and will also
support the identification of novel biomarkers for pulmonary fibrosis.

## Key facts

- **NIH application ID:** 10284461
- **Project number:** 1R21AR079765-01
- **Recipient organization:** MEDICAL UNIVERSITY OF SOUTH CAROLINA
- **Principal Investigator:** Peggi M Angel
- **Activity code:** R21 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $364,870
- **Award type:** 1
- **Project period:** 2021-09-27 → 2024-08-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10284461

## Citation

> US National Institutes of Health, RePORTER application 10284461, Development of proteomic-based ECM signatures for lung fibrosis (1R21AR079765-01). Retrieved via AI Analytics 2026-05-25 from https://api.ai-analytics.org/grant/nih/10284461. Licensed CC0.

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