[HTML][HTML] Accumulation of the Raf-1 kinase inhibitory protein (Rkip) is associated with Cep290-mediated photoreceptor degeneration in ciliopathies

CA Murga-Zamalloa, AK Ghosh, SB Patil… - Journal of Biological …, 2011 - ASBMB
Primary cilia regulate polarized protein trafficking in photoreceptors, which are dynamic and
highly compartmentalized sensory neurons of retina. The ciliary protein Cep290 modulates
cilia formation and is frequently mutated in syndromic and non-syndromic photoreceptor
degeneration. However, the underlying mechanism of associated retinopathy is unclear.
Using the Cep290 mutant mouse rd16 (retinal degeneration 16), we show that Cep290-
mediated photoreceptor degeneration is associated with aberrant accumulation of its novel …