Rare lung disease tissue

Strange Translational Research Lab

Charlton B. Strange, M.D.Charlie Strange, M.D.
Division of Pulmonary, Critical Care, Allergy and Sleep Medicine

Research Interests

The Strange laboratory works to improve detection, accurate diagnosis and therapy of rare lung diseases. Most of our current work seeks to determine the natural history of emphysema pathogenesis in alpha-1 antitrypsin deficiency (AATD). AATD was the first genetic disease associated with emphysema, but is a disease heavily influenced by environmental exposures, particularly cigarette smoking. Patients with different genotypes of SERPINA1 and with heterogeneous manifestations of lung disease including asthma, bronchiectasis, and emphysema have been studied in a series of NIH clinical trials. Peripheral blood mononuclear cell RNA, bronchoalveolar lavage cellular, proteomic and transcriptomic signatures are compared to clinical disease features to understand pathways that contribute to disease progression.

In alpha-1 antitrypsin deficiency, clinical trials with gene therapy for lung disease and siRNA therapy for liver disease are seeking to define novel pathways to improve clinical outcomes. Our understanding of the anti-inflammatory effects of alpha-1 antitrypsin has translated to a current trial of giving AAT augmentation to individuals with severe pancreatitis who are having a pancreatectomy with islet autotransplantation in an attempt to prevent the development of diabetes mellitus. In lymphangioleiomyomatosis (LAM), PDGF inhibition with imatinib mesylate seeks to define if this pathway is important in lung disease progression.

Our work requires close collaboration with the MUSC Clinical Translational Science Award (CTSA), support from the NEXUS center, frequent interaction with the MUSC IRB, significant biostatistical support, and close collaborations with many rare disease researchers and affected individuals across the world. Further information is available at alpha1research.org.


PubMed Collection

Senior Author Publications:

Senior Author PubMed Collection (senior author publications are most often associated with mentored projects)