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Title of Journal: Acta Neuropathol

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Abbravation: Acta Neuropathologica

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Springer-Verlag

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10.1007/978-3-319-45809-0_14

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1432-0533

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Neuronal and glial accumulation of α and βsynucl

Authors: Kyoko Suzuki Eizo Iseki Takashi Togo Akira Yamaguchi Omi Katsuse Kayoko Katsuyama Seiichi Kanzaki Kazumasa Shiozaki Chiaki Kawanishi Sumimasa Yamashita Yukichi Tanaka Shoji Yamanaka Yoshio Hirayasu
Publish Date: 2007/07/25
Volume: 114, Issue: 5, Pages: 481-489
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Abstract

A number of the lysosomal storage diseases that have now been characterized are associated with intralysosomal accumulation of lipids caused by defective lysosomal enzymes We have previously reported neuronal accumulation of both α and βsynucleins in brain tissue of a GM2 gangliosidosis mouse model Although αsynuclein has been implicated in several neurodegenerative disorders including Parkinson’s disease dementia with Lewy bodies and multiple system atrophy its functions remain largely unclear In our present study we have examined a cohort of human lipidosis cases including Sandhoff disease Tay–Sachs disease metachromatic leukodystrophy βgalactosialidosis and adrenoleukodystrophy for the expression of α and βsynucleins and the associated lipid storage levels The accumulation of αsynuclein was found in brain tissue in not only cases of lysosomal storage diseases but also in instances of adrenoleukodystrophy which is a peroxisomal disease αsynuclein was detected in both neurons and glial cells of patients with these two disorders although its distribution was found to be diseasedependent In addition αsynucleinpositive neurons were also found to be NeuNpositive whereas NeuNnegative neurons did not show any accumulation of this protein By comparison the accumulation of βsynuclein was detectable only in the pons of Sandhoff disease cases This differential accumulation of α and βsynucleins in human lipidoses may be related to functional differences between these two proteins In addition the accumulation of αsynuclein may also be a condition that is common to lysosomal storage diseases and adrenoleukodystrophies that show an enhanced expression of this protein upon the elevation of stored lipidsWe thank Dr K Ueda for his generous gift of antibodies and we are grateful to Dr Tatematsu and the late Dr Yokoi for providing additional materials The present study was supported in part by a Grant in Aid for Scientific Research No17790817 from the Japanese Society for the Promotion of Science

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