Ion Channels, Cortex Development & Disease
The Smith lab seeks to understand the role of neuronal activity in the development of the perinatal brain. We leverage classic neurobiology approaches and modern genetic tools to develop translational research insights, in a effort to treat early life ion channel diseases. Our training philosophy is to equip young scientists with skills to perform leading research and technology development, while also focusing on pressing issues in the scientific community.
What is a prenatal channelopathy?
A "Prenatal Channelopathy" is a developmental ion channel disease with a pathophysiological origin during the gestational period. The gestational brain is comprised of diverse and transient cell types, including dividing and newly differentiated cells. The mechanisms involved in prenatal channelopathies are different from those acting postnatally during ion channel diseases. For more info, see our recent review or research page.
Using the ferret to study brain development?
The domestic ferret (Mustela putorius furo) possess a folded neocortex and increased progenitor cell diversity. The developmentally tractable ferret brain therefore offers a promising platform for mechanistic testing of developmental diseases associated with gyrification deficits, such as prenatal channelopathies. Since small primates (marmosets) and mice both have smooth (lysencephalic) brains, alternative animal models such as the ferret are critical for studying brain folding. See recent work by Dr.Smith in Neuron and Nature utilizing these animals.
Human iPSC Models of ion channel diseases and therapeutics
Left, neurons differentiated from human induced pluripotent stems cells (iPSCs) from a child with a severe developmental ion channel disorder. Using iPSC's, we can understand the biological mechanisms of ion channel diseases, as well as rescue pathophysiological phenotypes. See Research page.
