title: The bacterial toxin CNF1 as a tool to induce retinal degeneration reminiscent of retinitis pigmentosa.
abstract: Retinitis pigmentosa (RP) comprises a group of inherited pathologies characterized by progressive photoreceptor degeneration. In rodent models of RP, expression of defective genes and retinal degeneration usually manifest during the first weeks of postnatal life, making it difficult to distinguish consequences of primary genetic defects from abnormalities in retinal development. Moreover, mouse eyes are small and not always adequate to test pharmacological and surgical treatments. An inducible paradigm of retinal degeneration potentially extensible to large animals is therefore desirable. Starting from the serendipitous observation that intraocular injections of a Rho GTPase activator, the bacterial toxin Cytotoxic Necrotizing Factor 1 (CNF1), lead to retinal degeneration, we implemented an inducible model recapitulating most of the key features of Retinitis Pigmentosa. The model also unmasks an intrinsic vulnerability of photoreceptors to the mechanism of CNF1 action, indicating still unexplored molecular pathways potentially leading to the death of these cells in inherited forms of retinal degeneration.
authors: Neuroscience Institute, Italian National Research Council (CNR), Pisa, 56124, Italy.
authors: Viviana
authors: V
authors: Guadagni
authors: Neuroscience Institute, Italian National Research Council (CNR), Pisa, 56124, Italy.|Accademia dei Lincei, Rome, 00165, Italy.
authors: Chiara
authors: C
authors: Cerri
authors: Department of Pharmacy, University of Pisa, Pisa, 56126, Italy.
authors: Ilaria
authors: I
authors: Piano
authors: Neuroscience Institute, Italian National Research Council (CNR), Pisa, 56124, Italy.
authors: Elena
authors: E
authors: Novelli
authors: Department of Pharmacy, University of Pisa, Pisa, 56126, Italy.
authors: Claudia
authors: C
authors: Gargini
authors: Istituto Superiore di Sanità, Rome, 00161, Italy.
authors: Carla
authors: C
authors: Fiorentini
authors: Neuroscience Institute, Italian National Research Council (CNR), Pisa, 56124, Italy.
authors: Matteo
authors: M
authors: Caleo
authors: Neuroscience Institute, Italian National Research Council (CNR), Pisa, 56124, Italy.
authors: Enrica
authors: E
authors: Strettoi
mesh_terms: D000818:Animals; D001427:Bacterial Toxins / Q000008:administration & dosage* / Q000633:toxicity*; D004195:Disease Models, Animal; D029968:Escherichia coli Proteins / Q000008:administration & dosage* / Q000633:toxicity*; D051379:Mice; D012160:Retina / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000139:chemically induced* / Q000473:pathology*
chemical_list: D001427:Bacterial Toxins; D029968:Escherichia coli Proteins; C060296:cytotoxic necrotizing factor type 1