ONGOING RESEARCH

The Welsh laboratory emphasizes three main areas.   The first is understanding the biology of cystic fibrosis (CF), a common lethal genetic disease.   We study the structure and function of the CF gene product, the CFTR Cl- channel, we are learning how CF-associated mutations disrupt the function and biosynthesis of the channel, and we are investigating the pathogenesis of lung disease.   We are focusing on the normal innate immune system and its disruption in CF.

Second, we are developing gene transfer as a potential treatment for CF and other genetic diseases.   The studies include basic vectorology, preclinical studies, and studies in humans.  

Third, we are studying the cellular and molecular biology of a novel class of non-voltage gated cation channels that includes the epithelial sodium channel (ENaC) and degenerins in C. elegans.    The work focuses on understanding how the subunits form a channel and how their activity is controlled.   We are especially interested in their sensory funciton, including mechanosensation and pain sensation.   We are investigating their physiology and model systems, in flies, and in transgenic and knockout mice.   Our goal is also to learn how normal and abnormal functions of these channels contribute to human diseases such as hypertension, neurodegeneration, and other neuronal abnormalities.

To see a more in-depth look at these three areas see descriptions below. There is also a description of the laboratory Core Facilities.