Ion-current-based proteomic profiling of the retina in a rat model of Smith-Lemli-Opitz syndrome.

ABSTRACT: Smith-Lemli-Opitz syndrome (SLOS) is one of the most common recessive human disorders and is characterized by multiple congenital malformations as well as neurosensory and cognitive abnormalities. A rat model of SLOS has been developed that exhibits progressive retinal degeneration and visual dysfunction; however, the molecular events underlying the degeneration and dysfunction remain poorly understood. Here, we employed a well-controlled, ion-current-based approach to compare retinas from the SLOS rat model to retinas from age- and sex-matched control rats (n = 5/group). Retinas were subjected to detergent extraction and subsequent precipitation and on-pellet-digestion procedures and then were analyzed on a long, heated column (75 cm, with small particles) with a 7-h gradient. The high an

SUBMITTER: Tu C 

PROVIDER: S-EPMC3861709 | biostudies-literature | 2013 Dec

REPOSITORIES: biostudies-literature