[HTML][HTML] Zebrafish and inherited photoreceptor disease: Models and insights

NCL Noel, WT Allison, IM MacDonald… - Progress in Retinal and …, 2022 - Elsevier
Photoreceptor dysfunctions and degenerative diseases are significant causes of vision loss
in patients, with few effective treatments available. Targeted interventions to prevent or …

Zebrafish models of photoreceptor dysfunction and degeneration

NCL Noel, IM MacDonald, WT Allison - Biomolecules, 2021 - mdpi.com
Zebrafish are an instrumental system for the generation of photoreceptor degeneration
models, which can be utilized to determine underlying causes of photoreceptor dysfunction …

Van Gogh-like 2 is essential for the architectural patterning of the mammalian biliary tree

M Raab, E Christodoulou, R Krishnankutty… - Journal of …, 2024 - Elsevier
Background & Aims In the developing liver, bipotent epithelial progenitor cells undergo
lineage segregation to form hepatocytes, which constitute the bulk of the liver parenchyma …

ARL13B, a Joubert syndrome-associated protein, is critical for retinogenesis and elaboration of mouse photoreceptor outer segments

TL Dilan, AR Moye, EM Salido… - Journal of …, 2019 - Soc Neuroscience
Mutations in the Joubert syndrome-associated small GTPase ARL13B are linked to
photoreceptor impairment and vision loss. To determine the role of ARL13B in the …

Notch inhibition promotes regeneration and immunosuppression supports cone survival in a zebrafish model of inherited retinal dystrophy

J Fogerty, P Song, P Boyd, SE Grabinski… - Journal of …, 2022 - Soc Neuroscience
Photoreceptor degeneration leads to irreversible vision loss in humans with retinal
dystrophies such as retinitis pigmentosa. Whereas photoreceptor loss is permanent in …

Ciliary genes arl13b, ahi1 and cc2d2a differentially modify expression of visual acuity phenotypes but do not enhance retinal degeneration due to mutation of cep290 …

EM Lessieur, P Song, GC Nivar, EM Piccillo, J Fogerty… - PloS one, 2019 - journals.plos.org
Mutations in the gene Centrosomal Protein 290 kDa (CEP290) result in multiple ciliopathies
ranging from the neonatal lethal disorder Meckel-Gruber Syndrome to multi-systemic …

Insights into photoreceptor ciliogenesis revealed by animal models

W Baehr, C Hanke-Gogokhia, A Sharif, M Reed… - Progress in retinal and …, 2019 - Elsevier
Photoreceptors are polarized neurons, with very specific subcellular compartmentalization
and unique requirements for protein expression and trafficking. Each photoreceptor contains …

Zebrafish models of human eye and inner ear diseases

B Blanco-Sánchez, A Clément, JB Phillips… - Methods in cell …, 2017 - Elsevier
Eye and inner ear diseases are the most common sensory impairments that greatly impact
quality of life. Zebrafish have been intensively employed to understand the fundamental …

Retinal degeneration and regeneration—Lessons from fishes and amphibians

D Ail, M Perron - Current pathobiology reports, 2017 - Springer
Abstract Purpose of Review Retinal degenerative diseases have immense socio-economic
impact. Studying animal models that recapitulate human eye pathologies aids in …

Insights gained from zebrafish models for the ciliopathy Joubert syndrome

TDS Rusterholz, C Hofmann… - Frontiers in …, 2022 - frontiersin.org
Cilia are quasi-ubiquitous microtubule-based sensory organelles, which play vital roles in
signal transduction during development and cell homeostasis. Dysfunction of cilia leads to a …