Ontology highlight
ABSTRACT:
SUBMITTER: Jones W
PROVIDER: S-EPMC6361150 | biostudies-literature | 2019 Jan
REPOSITORIES: biostudies-literature
Jones Wendell W Rodriguez Juan J Bassnett Steven S
Disease models & mechanisms 20190125 1
Fibrillin is an evolutionarily ancient protein that lends elasticity and resiliency to a variety of tissues. In humans, mutations in fibrillin-1 cause Marfan and related syndromes, conditions in which the eye is often severely affected. To gain insights into the ocular sequelae of Marfan syndrome, we targeted <i>Fbn1</i> in mouse lens or non-pigmented ciliary epithelium (NPCE). Conditional knockout of <i>Fbn1</i> in NPCE, but not lens, profoundly affected the ciliary zonule, the system of fibril ...[more]