Summary
Studies using the Golgi method were performed on neocortical and cerebellar tissues from a 9-month-old cat with a history of progressive neurological deterioration and a subsequently demonstrated deficiency of the lysosomal enzyme α-d-mannosidase in both neural and non-neural tissues. Many cortical pyramidal neurons demonstrated morphological alterations involving formation of abnormal oplargements (meganeurites) at the axon hillock-initial segment area, abnormal sprouting of neurites (secondary neurites) in this same region, and various types of dendritic changes, such as formation of focal enlargements, thinning, and spine loss. Many nonpyramidal neurons also were abnormal but displayed only dendritic changes similar to those seen in pyramidal neurons. Cerebellar Purkinje cells displayed dendritic systems marked by focal swellings and often demonstrated one or more enlargements within axons (axonal spheroids) at some distance from otherwise normal-appearing cell bodies.
Feline mannosidosis appears to be another of the lysosomal storage diseases in which highly specialized morphological changes accompany storage of unmetabolized substrate and contribute to the pathogenesis of the disease process.
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References
Baker HJ, Lindsey JR, McKhann GM, Farrell DF (1971) Neuronal GM1 gangliosidosis in a Siamese cat with β-galactosidase deficiency. Science 174:838–839
Blackwood W, Corsellis JAN (1976) Greenfield's neuropathology, 3rd edn. Arnold, London
Blakemore WF, Uygur P (1980) A case of feline mannosidosis. Clinical and histopathological findings (in prep.)
Braak H, Goebel HH (1979) Pigmentoarchitectonic pathology of the isocortex in juvenile ceroid lipofusinosis. Axonal enlargements in layer IIIab and cell loss in layer V. Acta Neuropathol (Berl) 46:79–83
Burditt LJ, Chorlai K, Hirani S, Nugen PG, Winchester B, Blakemore WF (1980) A case of feline mannosidosis: Biochemical studies. Biochem J (in press)
Cork L, Munnell JF, Lorenz MD, Murphy JV, Baker HJ, Rattazzi MC (1977) GM2 ganglioside lysosomal storage disease in cats with β-hexosaminidase deficiency. Science 196:1014–1017
Cork L, Munnell JF, Lorenz MD (1978) The pathology of feline GM2 gangliosidosis. Am J Pathol 90:723–734
Ferrer I, Arbizu T, Peña J, Serra JP (1980) A Golgi and ultrastructural study of a dominant form of Kuf's disease. J. Neurol 222:183–190
Ghatak NR, Fleming DF, Hinman A (1977) Neuropathology of Sanfilippo syndrome. Ann Neurol 2:161–166
Jack JJB, Noble D, Tsien RW (1975) Electric current flow in excitable cells. Clarendon Press, Oxford
Jolly RD (1971) The pathology of the central nervous system in pseudolipidosis of angus calves. J Pathol 103:113–121
Jolly RD, Thompson KG (1978) The pathology of bovine mannosidosis. Vet Pathol 15:141–152
Kjellman B, Gamstorp I, Brun A, Öckerman P, Palmgren B (1969) Mannosidosis. A clinical and histopathological study. J Pediatr 75:366–373
Purpura DP (1978) Ectopic dendritic growth in mature pyramidal neurones in human ganglioside storage disease. Nature 276:520–521
Purpura DP, Baker HJ (1977) Neurite induction in mature cortical neurones in feline GM1 ganglioside storage disease. Nature 226:553–554
Purpura DP, Baker HJ (1978) Meganeurites and other aberrant processes of neurons in feline GM1 gangliosidosis. Brain Res 143:13–26
Purpura DP, Suzuki K (1976) Distortion of neural geometry and formation of aberrant synapses in neuronal storage disease. Brain Res 116:1–21
Purpura DP, Pappas GD, Baker HJ (1978) Fine structure of meganeurites and secondary growth processes in feline GM1 gangliosidosis. Brain Res 143:1–12
Purpura DP, Highstein SM, Karabelas AB, Walkley SU (1980) Intracellular recording and HRP staining of cortical neurons in feline ganglioside storage disease. Brain Res 181:446–449
Rall W (1977) Core conductor theory and cable properties of neurons. In: Brookhart JM, Mountcastle VB (eds) Handbook of physiology, sect 1. The nervous system, vol 1, part 1. Waverly Press, Baltimore, pp 39–97
Sung JH, Hayano M, Desnick RJ (1977) Mannosidosis. Pathology of the nervous system. J Neuropathol Exp Neurol 36:807–820
Walkley SU, Baker HJ, Purpura DP (1980) Morphological changes in feline GM1 gangliosidosis. A Golgi study. In: Rose FC, Behan PO (eds) Animal models in neurological disease. Pitmans Medical, London (in press)
Walkley SU, Purpura DP, Baker HJ (1979) Development of abnormal neuron morphology in feline gangliosidosis. Soc Neurosc Abstr 5:520
Williams RS, Lott IT, Ferrante RJ, Caviness VS (1977) The cellular pathology of neuronal lipofuscinosis. Arch. Neurol 34:298–305
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This work was supported by Research Grants NS-07512 and NS-06232 from the National Institutes of Health
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Walkley, S.U., Blakemore, W.F. & Purpura, D.P. Alterations in neuron morphology in feline mannosidosis. Acta Neuropathol 53, 75–79 (1981). https://doi.org/10.1007/BF00697187
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DOI: https://doi.org/10.1007/BF00697187