In Search of Mouse Models for Exfoliation Syndrome.

ABSTRACT:

Purpose

Exfoliation syndrome (XFS) is a systemic connective tissue disorder with elusive pathophysiology. We hypothesize that a mouse model with elastic fiber defects caused by lack of lysyl oxidase like 1 (LOXL1 encoded by Loxl1), combined with microfibril deficiency due to Fbn1 mutation (encoding fibrillin-1, Fbn1^C1041G/+) will display ocular and systemic phenotypes of XFS.

Methods

Loxl1^-/- was crossed with Fbn1^C1041G/+ to create double mutant (dbm) mice. Intraocular pressure (IOP), visual acuity (VA), electroretinogram (ERG), and biometry were characterized in 4 genotypes (wt, Fbn1^C1041G/+, Loxl1^-/-, dbm) at 16 weeks of age. Optic nerve (ON) area was measured by ImageJ, and axon counting was achieved by AxonJ. Deep wh

SUBMITTER: Kuchtey RW 

PROVIDER: S-EPMC11486597 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature