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OVINE CEROID‐LIPOFUSCINOSIS: A MODEL OF BATTEN'S DISEASE
Author(s) -
JOLLY R. D.,
JANMAAT A.,
WEST D. M.,
MORRISON I.
Publication year - 1980
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1111/j.1365-2990.1980.tb00290.x
Subject(s) - batten disease , neuronal ceroid lipofuscinosis , neuropathology , lipofuscin , disease , lysosomal storage disease , biology , pathogenesis , pathology , medicine , immunology
Jolly R.D., Janmaat A., West D.M. & Morrison I. 1980 Neuropathology and Applied Neurobiology 6,195–209 Ovine ceroid‐lipofuscinosis: a model of Batten's disease An inherited neurological disease of sheep was characterized by the intracellular accumulation of autofiuorescent lipopigments in neurones and a wide variety of other cells within the body. The staining, fluorescent, ultrastruc‐tural and physical characteristics of the storage material were similar to those found in a heterogeneous group of storage diseases of children known as Batten's disease or the ceroid‐lipofuscinoses. The ovine disease did not exactly fit any of the main human entities, but had features in common with both the late infantile and juvenile forms. It was concluded that this was a useful model for studying the pathogenesis of this type of storage disease and for therapeutic trials. A flock of sheep is maintained for this purpose.