Harmonin (Ush1c) is required in zebrafish Muller glial cells for photoreceptor synaptic development and function.

ABSTRACT: Usher syndrome is the most prevalent cause of hereditary deaf-blindness, characterized by congenital sensorineural hearing impairment and progressive photoreceptor degeneration beginning in childhood or adolescence. Diagnosis and management of this disease are complex, and the molecular changes underlying sensory cell impairment remain poorly understood. Here we characterize two zebrafish models for a severe form of Usher syndrome, Usher syndrome type 1C (USH1C): one model is a mutant with a newly identified ush1c nonsense mutation, and the other is a morpholino knockdown of ush1c. Both have defects in hearing, balance and visual function from the first week of life. Histological analyses reveal specific defects in sensory cell structure that are consistent with these behavioral phenotypes

SUBMITTER: Phillips JB 

PROVIDER: S-EPMC3209648 | biostudies-literature | 2011 Nov

REPOSITORIES: biostudies-literature