Cho Research
Adriana Mantegazza, PhD

Contact

Name: Seo-Hee Cho, PhD
Position: Director, Vision Research Core Facility

1020 Locust Street
JAH 425G
Philadelphia, PA 19107

Telephone: 215-955-2129

My lab is interested in the organ growth regulation mediated by Hippo-Yap signaling, and novel disease mechanisms underlying the early-onset, blinding disease caused by mutations in Crumbs homolog 1 (Crb1) gene.

Research Projects

Understanding Hippo-Yap signaling-mediated organ size regulation in ocular development

Hippo-Yap signaling plays critical functions in various developmental and injury events such as organ size control and cancers. We are investigating how Yap-Tead-mediated transcription activity and upstream suppressor, Lats1/2, orchestrate the development of eye tissues by regulating cellular processes such as proliferation, death, fate choice and maintenance of apico-basal polarity. 

Elucidating pathological mechanisms of the early-onset blindness in mouse models

Leber congenital amaurosis 8 (LCA8) is the most severe, non-treatable, early-onset blinding disease that is caused by recessive mutations in Crumbs homolog 1 (Crb1) gene. About 20% of children attending schools for the blind around world are affected by LCA. Recently we generated mouse model of LCA8 by conditional gene knock-out of Crb1 and its paralog, Crb2, which recapitulated crucial features of human LCA8 pathology. These include temporarily thickened, delaminated retina and early-onset visual loss at the eye opening stage. Our current research focuses on characterizing cellular and molecular defects in the mutant retinal progenitor cells, which undergo defective interkinetic nuclear migration that may alter cellular processes involved in cell proliferation, growth, apico-basal polarity and actomyosin cytoskeleton regulation. In addition, we investigate the outcome of the perturbed intracellular signaling cascades that are mediated by Hippo-Yap, TSC1/2-mTOR and RhoA-ROCK pathways. Our goal is to delineate retinal progenitor-specific cellular and molecular mechanisms underlying LCA8 pathologies using genetic and pharmacological approaches.