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PMID:15565184

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Citation

Seigel, GM, Wagner, J, Wronska, A, Campbell, L, Ju, W and Zhong, N (2005) Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis. Eye (Lond) 19:1306-12

Abstract

Accumulation of autofluorescent storage material in the CNS is a hallmark of neuronal ceroid lipofuscinosis (NCL, Batten disease). Since the retina is generally the first CNS target affected in NCL and could serve as a means to assess early disease progression as well as potential therapeutic responses, we followed the course of postnatal retinal pathology in tissues from the CLN8 (mnd) mouse model of NCL.

Links

PubMed Online version:10.1038/sj.eye.6701770

Keywords

Aging/pathology; Animals; Cell Death; Disease Models, Animal; Disease Progression; Fluorescence; Hypertrophy/pathology; In Situ Nick-End Labeling; Inclusion Bodies/pathology; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Microscopy, Fluorescence; Neuronal Ceroid-Lipofuscinoses/pathology; Retinal Degeneration/pathology; Retinal Ganglion Cells/pathology

Significance

Annotations

Gene product Qualifier GO ID GO term name Evidence Code with/from Aspect Notes Status


See also

References

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