Glial cells and pharmacological targets in Sandhoff disease
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- Ogawa Yasuhiro
- Department of Pharmacology, Meiji Pharmaceutical University
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- Sakuraba Hitoshi
- Department of Clinical Genetics, Meiji Pharmaceutical University
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- Oishi Kazuhiko
- Department of Pharmacology, Meiji Pharmaceutical University
Bibliographic Information
- Other Title
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- Sandhoff病の病態とグリア細胞,その創薬薬理
- Sandhoffビョウ ノ ビョウタイ ト グリア サイボウ,ソノ ソウヤク ヤクリ
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Abstract
<p>Sandhoff disease (SD) is a genetic disorder caused by a mutation in the β-hexosaminidase B (HexB) gene in humans. This results in the massive accumulation of GM2 gangliosides in the nervous system, causing progressive neurodegeneration. The symptoms of SD include muscle weakness, seizures, and mental illness;along with loss of muscle coordination, vision, and hearing. In the most severe form, the onset begins during early infancy, and death usually occurs within 3-5 years of age. The established animal model, Hexb-deficient (Hexb−/−) mouse, shows abnormalities that resemble the severe phenotype found in human infants. We have previously reported that activated microglia causes astrogliosis in Hexb−/− mouse at the early stage of development that can be ameliorated via immunosuppression. Moreover, within the cerebral cortices of Hexb−/− mouse, reactive astrocytes were found to express adenosine A2A receptors in later inflammatory phases. Inhibiting this receptor with istradefylline decreases the number of activated microglial cells and inflammatory cytokines/chemokines. Thus, we underline the importance of the astrocytic A2A receptor as a sensor, in regulating microglial activation in the late phase of inflammation.</p>
Journal
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- Folia Pharmacologica Japonica
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Folia Pharmacologica Japonica 156 (4), 235-238, 2021
The Japanese Pharmacological Society
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Details 詳細情報について
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- CRID
- 1390570022236155136
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- NII Article ID
- 130008060060
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- NII Book ID
- AN00198335
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- ISSN
- 13478397
- 00155691
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- NDL BIB ID
- 031603509
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- PubMed
- 34193703
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
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- Abstract License Flag
- Disallowed