Summary
A method for the measurement of autofluorescence emission spectra of intraneuronal lipopigment in tissue sections has been applied to specimens from dogs and sheep with forms of neuronal ceroid-lipofuscinosis (NCL). The characteristics of an emission spectrum probably reflect the composition of the lipopigment, and the results are compared with those previously reported from human NCLs and lipofuscin in non-diseased elderly human brains. Lipopigment in the animal NCLs differed from lipofuscin in the non-diseased human brains, but no differences could be demonstrated between the spectra from animal and human NCLs. These findings support the use of the animal diseases as models for research into the pathogenesis and treatment of human NCLs. Both human and animal NCLs would be more accurately designated as the “ceroidoses”.
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References
Armstrong D, Neville HE, Siakotos AN, Wilson B, Wehling C, Koppang N (1980) Morphological and biochemical abnormalities in a model of retinal degeneration: canine ceroid-lipofuscinosis (CCL). Neurochem Int 1:405, 426
Armstrong D, Siakotos A, Koppang N, Connole E (1978) Studies on the retina and the pigment epithelium in hereditary canine ceroid lipofuscinosis. 1. The distribution of enzymes in the whole retina and pigment epithelium. Invest Ophthalmol Visual Sci 17:608–617
Bourne GH (1973) Lipofuscin. In: Ford DH (ed) Progress in brain research, vol 40. Elsevier, New York, pp 187–201
Cummings JF, de Lahunta A (1977) An adult case of canine neuronal ceroid-lipofuscinosis. Acta Neuropathol (Berl) 39:43–51
Dowson JH, Harris SJ (1981) Quantitative studies of the autofluorescence derived from neuronal lipofuscin. J Microscopy 123:249–258
Dowson JH (1982a) Neuronal lipofuscin accumulation in ageing and Alzheimer dementia: A pathogenic mechanism? Br J Psychiat 140:142–148
Dowson JH (1982b) The evaluation of autofluorescence emission spectra derived from neuronal lipopigment. J Microscopy (in press)
Gadoth N, O'Croinin P, Butler IJ (1975) Bone marrow in the Batten-Vogt syndrome. J Neurol Sci 25:197–203
Goebel HH, Schulz F (1979) The ultrastructural variability of non-specific lipopigments. Acta Neuropathol (Berl) 48:227–230
Goebel HH, Zeman W, Patel UK, Pullarkat RK, Lenard HG (1979) On the ultrastructural diversity and essence of residual bodies in neuronal ceroid-lipofuscinosis. Mech Ageing Develop 10:53–70
Greenwood RS, Nelson JS (1978) Atypical neuronal ceroid-lipofuscinosis. Neurology 28:710–717
Jolly RD, Janmaat A, West DM, Morrison I (1980) Ovine ceroid-lipofuscinosis: a model of Batten's disease. Neuropathol Appl Neurobiol 6:195–209
Koppang N (1973) Canine ceroid-lipofuscinosis — a model for human neuronal ceroid-lipofuscinosis and ageing. Mech Ageing Develop 2:421–445
Lake BD, Cavanagh NPC (1978) Early-juvenile Batten's disease — a recognisable sub-group distinct from other forms of Batten's disease. J Neurol Sci 36:265–271
Schröder R (1980) The lipopigments in human brain tissue necroses. Acta Neuropathol (Berl) 52:141–145
Siakotos AN (1974) Procedures for the isolation of brain lipopigments: ceroid and lipofuscin. Methods Enzymol 31:478–485
Siakotos AN, Armstrong D, Koppang N,Connole E (1978) Studies on the retina and the pigment epithelium in hereditary canine ceroid lipofuscinosis. Invest Ophthalmol Visual Sci 17:618–633
Vandervelde M, Fatzer R (1980) Neuronal ceroid-lipofuscinosis in older dachshunds. Vet Pathol 17:686–691
Williams RS, Lott IT, Ferrante RJ, Caviness US (1977) The cellular pathology of neuronal ceroid-lipofuscinosis. Arch Neurol 34:298–305
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Dowson, J.H., Armstrong, D., Koppang, N. et al. Autofluorescence emission spectra of neuronal lipopigment in animal and human ceroidoses (ceroid-lipofuscinoses). Acta Neuropathol 58, 152–156 (1982). https://doi.org/10.1007/BF00691656
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DOI: https://doi.org/10.1007/BF00691656