Conway Lab

The research laboratory of Simon Conway, PhD, is focused on examination and manipulation of the molecular regulatory mechanisms that govern cardiopulmonary development and disease. The lab specializes in using transgenic mouse modeling approaches to understand pathogenesis. With these models, the Conway Lab seeks to identify the mechanisms and cell lineages responsible for disease development and transgenically and/or pharmacologically correct the various structural and functional defects.

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Active Research

Over the last 19 years, the Conway Lab has primarily focused on the pathogenesis of transcription factors Pax3/7 and the transforming growth factor-beta (TGFβ) superfamily mediated cardiac neural crest (NC)-associated congenital conotruncal heart defects.

Conotruncal heart defects (CHDs) result from a failure of the aorta and pulmonary trunks of the outflow tract (OFT) to become separate blood vessels. These defects are frequently associated with endocardial cushion/valve anomalies, and mutant mice exhibiting these CHDs often exhibit bradycardia and die in utero. Furthermore, lack of NC-mediated OFT septation often results in respiratory failure and abnormal lung maturation. Through the use of both physiological hyperoxia and transgenic approaches, the lab is testing whether initial suppression of the TGFβ signaling apparatus, as well as loss of key downstream TGFβ superfamily effectors, underlie lung malformations.

Recently, the lab has become interested in the cardiopulmonary roles of the TGFβ-responsive Periostin and TGFBI matricellular proteins. Significantly, the lab has shown that a partial Periostin element is uniquely capable of driving reporter expression in activated cardiac myofibroblasts, providing a unique in vivo tool for genetic manipulation of activated injury-site fibroblasts. Additionally, the lab generated Periostin and TGFBI knockout mice to test their functional requirement in heart and lung maturation, their responses to injury and their roles during multi-organ homeostasis and fibrosis.

Studies conducted in the Conway Lab contribute significantly to the current understanding of cardiopulmonary defect pathogenesis and seek to use basic research to uncover novel therapeutic approaches for the diagnosis and mitigation of life-threatening diseases routinely seen in the clinic.

Research Funding

Molecular Mechanisms of Neural Crest-Related Congenital Heart Defects

Goals: To examine how Pax3 transcription factor regulates cardiac neural crest morphogenesis and determine the underlying mechanism of in utero heart failure in Pax3 nulls using systemic null, hypomorphic and lineage-restricted conditional mouse mutant models.

Recent Publications

For a full list of Conway’s publications, find him on PubMed.

Faculty Research Team

15408-Conway, Simon Simon J. Conway, PhD

Simon J. Conway, PhD

Professor of Pediatrics