Sufactant Protein C in the Diagnosis and Treatment of Interstitial Lung Disease
Background
- Pulmonary surfactant is a heterogeneous mixture of phospholipids and proteins that lines the alveolar surface of the lungs.
- Surfactant protein C (SP-C) in a 34-35 amino acid peptide expressed specifically in type II epithelial cells in the alveolus is known to influence surface properties of lung phospholipids to enhance surfactant functions.
- Recently, insights into various roles of SP-C have been provided by studies in patients with selective deficiency of SP-C gene or dominantly inherited mutations in the SP-C gene.
- For example, a dominantly inherited mutation in SP-C gene is assosciated with interstitial lung disease (ILD).
- ILD includes a heterologous collection of uncommon disorders detected in individuals who present with progressive lung disorders associated with frequent pulmonary infections, exercise limitations, tachypnea and shortness of breath.
- In general, ILD is associated in alveolar inflammation, pulmonary infiltration with monocytes and macrophages, progressive loss of alveolar structure and pulmonary fibrosis.
Description of Current Technology
Dr. Jeffrey Whitsett of the Cincinnati Children's Research Foundation has demonstrated that genetic ablation of SP-C in mice causes a progressive severe pulmonary fibrosis, epithelial cell dysplasia in conducting airways, emphysema, alveolar vascular remodeling and right heart failure. Taken together with findings in humans bearing dominantly inherited gene mutations in SP-C support the concept that a deficiency of proSP-C or SP-C per se might also cause pulmonary disease. These pathologies include idiopathic pulmonary fibrosis (IPF), desquamated interstitial pneumonitis (DIP) and other forms of ILD. Thus, administration of SP-C via aerosol or instillation by other pharmacological means represents a novel approach to the treatment of ILD. Likewise, SP-C gene replacement therapy opens up new avenues to treatment of these disorders. Furthermore, gene array analysis offers a promising diagnostic approach in populations of patients suspected of ILD.
Objective
We are currently seeking a corporate partner to provide funding for proof of concept studies, preferably under an option agreement and/or to enter into a license agreement for the commercial development of a meaningful therapy utilizing this technology. U.S. and international patent applications have been filed.
Contact
To review further data or other confidential information, please contact:
Joseph D. Fondacaro, PhD Director
Office of Intellectual Property & Venture Development Cincinnati Children's Research Foundation
Mail Location 7032
3333 Burnet Avenue
Cincinnati, Ohio 45229-3039
Phone: 513-636-7695
Fax: 513-636-8453
Email: jdfonda@cchmc.org
Related Study Information
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