NCL Description for Cocker Spaniels

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NCL Description for Cocker Spaniels Age of onset of clinical signs: 1.5 - 6 years Age of death or euthanasia: 1.5 - 6 years Abnormalities often observed by the owner: Mental changes: Aggression, irritability, dementia Changes in gait and posture: progressive difficulty walking; weakness and uncoordinated movement. Visual abnormalities: blindness may be present in some cases Seizures/convulsions: reported in some cases Other changes: Jaw champing, head tremors, emaciation Abnormalities observed upon clinical examinations: Clinical neurologic changes: Gait abnormalities, weakness, uncoordinated movement (hypermetric ataxia, proprioceptive deficits, exaggerated spinal reflexes) Clinical ophthalmic changes: ophthalmic changes have been reported Visual abnormalities: blindness has been reported Retinal changes: retinal atrophy has been reported Electroretinography (ERG): not described in reported cases Other clinical findings: none reported Histopathology Brain: Yellow-brown granules were present in neuronal cytoplasm of some neurons in the brain and spinal cord. Storage granules were most abundant in the spinal cord and cerebellum. Cells in the cerebral cortex, brain stem, and Purkinje cells were less affected. Affected neurons were swollen, and often had an eccentric nucleus due to displacement by accumulation of granules. These granules exhibited yellow-green autofluorescence and staining patterns consistent with ceroid and lipofuscin. Degenerative changes within the CNS were also described (neuronal necrosis, Wallerian degeneration, and axonal dystrophy). Eyes: mild irregular loss of photoreceptor cells; massive accumulation of fluorescent material in the retinal pigmented epithelium. Other organs and structures: Storage product also accumulated within the smooth muscle cells of the intestines, pancreas, urinary bladder, and walls of small arteries. Mode of inheritance: Autosomal recessive inheritance is suspected. Gene containing mutation: Unknown References: Minatel L, et al. Ceroid-lipofuscinosis in a Cocker Spaniel dog. Veterinary Pathology (2000); 37:488-490. Jolly RD, et al. Generalised ceroid-lipofuscinosis and brown bowel syndrome in Cocker spaniel dogs. New Zealand Veterinary Journal (1994); 42:236-239. Nimmo JS, et al. Neuronal and Generalized Ceroid-Lipofuscinosis in a Cocker Spaniel. Veterinary Pathology (1982); 19:623-628. .

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Formation of Lipofuscin-Like Autofluorescent Granules in the Retinal Pigment Epithelium Requires Lysosome Dysfunction
Retinal Cell Biology Formation of Lipofuscin-Like Autofluorescent Granules in the Retinal Pigment Epithelium Requires Lysosome Dysfunction Cristina Escrevente,1 Ana S. Falcão,1 Michael J. Hall,2 Mafalda Lopes-da-Silva,1 Pedro Antas,1 Miguel M. Mesquita,1 Inês S. Ferreira,1 M. Helena Cardoso,1 Daniela Oliveira,1 Ana C. Fradinho,1 Thomas Ciossek,3 Paul Nicklin,3 Clare E. Futter,2 Sandra Tenreiro,1 and Miguel C. Seabra1,2 1iNOVA4Health, CEDOC – Chronic Diseases Research Center, NOVA Medical School, Universidade Nova de Lisboa, Lisboa, Portugal 2UCL Institute of Ophthalmology, London, United Kingdom 3Research Beyond Borders, Boehringer Ingelheim, Biberach, Germany Correspondence: Miguel C. Seabra, PURPOSE. We aim to characterize the pathways required for autofluorescent granule (AFG) CEDOC - NOVA Medical School, formation by RPE cells using cultured monolayers. Universidade Nova de Lisboa, 1169-056 Lisboa, Portugal; METHODS. We fed RPE monolayers in culture with a single pulse of photoreceptor outer [email protected]. segments (POS). After 24 hours the cells started accumulating AFGs that were compa- rable to lipofuscin in vivo. Using this model, we used a variety of light and electron Received: January 24, 2021 microscopical techniques, flow cytometry and Western blot to analyze the formation of Accepted: June 29, 2021 Published: July 27, 2021 AFGs. We also generated a mutant RPE line lacking cathepsin D by gene editing. Citation: Escrevente C, Falcão AS, RESULTS. AFGs seem to derive from incompletely digested POS-containing phagosomes Hall MJ, et al. Formation of and after 3 days are surrounded by a single membrane positive for lysosome markers. We lipofuscin-like autofluorescent show by various methods that lysosome-phagosome fusion is required for AFG formation, granules in the retinal pigment and that impairment of lysosomal pH or catalytic activity, particularly cathepsin D activity, epithelium requires lysosome enhances AF accumulation.
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