BLR-200 demonstrates unique mechanism blocking activation of scar-forming cells in systemic sclerosis models

NORTH CHICAGO, Ill., April 30, 2026 /PRNewswire/ -- Rosalind Franklin University of Medicine and Science announced today that BLR Bio, a company in its Helix 51 incubator, presented new data this week at the 2026 British Society of Rheumatology conference in Glasgow, Scotland, highlighting the potential of its investigational drug BLR-200 for the treatment of systemic sclerosis (scleroderma).

"Our goal is to provide relief and healing to patients with this terrible disease," said Dr. Bruce Riser, CEO of BLR Bio and adjunct professor at RFU's Center for Cancer Cell Biology, Immunology and Infection. "We are encouraged by BLR-200's ability to interrupt key biological pathways that drive fibrosis, or tissue scarring, in scleroderma."

Systemic sclerosis is a rare, chronic autoimmune-based disease characterized by fibrosis and vascular abnormalities affecting the skin, joints and internal organs, especially the lungs, heart and kidneys, which account for most disease-related deaths. Early symptoms often include Raynaud's phenomenon, skin thickening, joint pain, and gastrointestinal issues. The disease disproportionately affects women (approximately 80% of patients) and can lead to significant disability, disfigurement, pain and fatigue. The World Scleroderma Foundation estimates that about 300,000 Americans are living with some form of scleroderma, with global figures ranging from 1.5 million to 2.5 million people. There are no FDA-approved therapies for scleroderma of the skin.

BLR Bio's newly presented data serves to further elucidate both the mechanism of action and efficacy of BLR-200 in a bleomycin-induced in vivo model of systemic sclerosis. Using single-cell RNA sequencing and UMAP analysis of total skin fibroblasts — cells with the potential to drive scarring — bleomycin induced a unique population expressing profibrotic markers known as engrailed-1 and COL8A1. Treatment with BLR-200 effectively eliminated this cell population, with a corresponding absence of skin thickening and fibrosis in the treated preclinical models.

"It is exciting to learn that BLR-200's downstream effect is prevention of activation of engrailed-1/COL8A1-expressing fibroblasts, a highly specific group of scar-forming cells," Dr. Riser said. "Recent studies have shown that an equivalent skin fibroblast population is present in human scleroderma and is responsible for the overproduction of collagen that characterizes the disease. This further differentiates our therapy from that of others. Also, we know that BLR-200 modulates YAP nuclear translocation — a cellular response to stiff matrices (scarring). This continues to be a critical target in developing therapies for fibro-inflammatory disease.

The study was conducted in collaboration with leading researchers and their institutions, including: Dr Andrew Leask, University of Saskatchewan, Saskatoon, Canada; Dr. Richard Stratton, Centre for Rheumatology, UCL Division of Medicine, Hampstead, London, UK; Dr James Varani, Department of Pathology, University of Michigan, Ann Arbor; and the BLR Bio laboratory at RFU. 

BLR Bio's in vivo data aligns with recent published research showing that extracellular matrix deposition — the buildup of fibrotic tissue — in human scleroderma skin originates from myofibroblasts expressing these same markers. Together, the data help bridge preclinical models and human disease, supporting the treatment potential for BLR-200. 

BLR Bio also presented data on its research on CCN proteins — signaling modulators in development, wound healing, and tissue repair — as potential non-invasive biomarkers for systemic sclerosis. The studies revealed distinct serum patterns of CCN3 and CCN2 between early versus late-stage patients and healthy controls. Notably, CCN3 alone showed promise as a biomarker to differentiate scleroderma patients from healthy individuals.

The company continues to advance its research on novel peptide-based therapies targeting fibrotic diseases and cancer. Its primary laboratories are based in RFU's Helix 51 incubator, with collaborations including RFU, the University of Michigan, University of Saskatchewan, and the Mario Negri Institute and others.

"Scleroderma is a devastating autoimmune disease with few treatment options," said RFU Interim Executive Vice President for Research Dr. Janice Urban. "We are hopeful that Dr. Riser's expertise and BLR Bio's innovative approach will lead to meaningful advances that improve both quality and length of life for patients."

About Rosalind Franklin University
Rosalind Franklin University of Medicine and Science embodies the spirit of inquiry and excellence modeled by its namesake Dr. Rosalind Franklin, whose Photo 51 was crucial to solving the structure of DNA. Recognized for its research in areas including neuroscience, brain-related diseases, inherited disorders, diabetes, obesity, and gait and balance, RFU encompasses the Chicago Medical School, College of Health Professions, College of Nursing, College of Pharmacy, School of Graduate and Postdoctoral Studies and the Dr. William M. Scholl College of Podiatric Medicine. Learn more at rosalindfranklin.edu.

About BLR Bio
Learn more at blrbio.com.

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