Abstract
The lysosome is a key catabolic organelle involved in many processes and its correct function is essential for health. In the retina it is crucial for phagocytosis of spent photoreceptor segments by the retinal pigmented epithelium. The zebrafish tpp1 mutant and cln3 morphant knockdown are both deficient in key lysosomal proteins. Both have a characteristic retinal phenotype during development but it is not clear is the phenotype is caused by abnormal developmental or degeneration. We will characterise the function of Tpp1 and Cln3 during retinal development, and determine any epistatic relationship.
References
Mahmood F, Fu S, Cooke J, Wilson SW, Cooper JD, Russell C. A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation. Brain. 2013; 136; 1488-1507
Wager K, Zdebik AA, Fu S, Cooper JD, Harvey RJ, Russell C. Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease). PLoS One. 2016;11(6):e0157365.
Zdebik AA, Mahmood F, Stanescu HC, Kleta R, Bockenhauer D, Russell C. Epilepsy in kcnj10 morphant zebrafish assessed with a novel method for long-term EEG recordings. PLoS One. 2013;8(11):e79765.
Gestri G, Bazin-Lopez N, Scholes C, Wilson SW. Cell Behaviors during Closure of the Choroid Fissure in the Developing Eye Frontiers in Cellular Neuroscience 2018; 12: 42
Chen S, Reichert S, Singh C, Oikonomou G, Rihel J*, and Prober DA* (2017).
Light-dependent regulation of sleep/wake states by prokineticin 2 in zebrafish.
Neuron. http://dx.doi.org/10.1016/j.neuron.2017.06.001. *co-corresponding