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Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse

Title
Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse
Authors
Bin Hong, YoungKim, Eun YoungJung, Sung-Chul
Ewha Authors
정성철
SCOPUS Author ID
정성철scopus
Issue Date
2006
Journal Title
JOURNAL OF KOREAN MEDICAL SCIENCE
ISSN
1011-8934JCR Link
Citation
vol. 21, no. 4, pp. 733 - 738
Keywords
Gaucher diseaseglucosylceramidaseglucocerebrosidasemodelsanimalmicebraincytokinesnitric oxide
Publisher
KOREAN ACAD MEDICAL SCIENCES
Indexed
SCI; SCIE; SCOPUS; KCI WOS scopus
Abstract
Gaucher disease is caused by a deficiency of glucocerebrosidase. Patients with Gaucher disease are divided into three major phenotypes: chronic nonneuronopathic, acute neuronopathic, and chronic neuronopathic, based on symptoms of the nervous system, the severity of symptoms, and the age of disease onset. The characteristics of patients with acute neuronopathic- and chronic neuronopathic-type Gaucher disease include oculomotor abnormalities, bulbar signs, limb rigidity, seizures and occasional choreoathetoid movements, and neuronal loss. However, the mechanisms leading to the neurodegeneration of this disorder remain unknown. To investigate brain dysfunction in Gaucher disease, we studied the possible role of inflammation in neurodegeneration during development of Gaucher disease in a mouse model. Elevated levels of the proinflammatory cytokines, IL-1 alpha, IL-1 beta, IL-6, and TNF-alpha, were detected in the fetal brains of Gaucher mice. Moreover, the levels of secreted nitric oxide and reactive oxygen species in the brains of Gaucher mice were higher than in wild-type mice. Thus, accumulated glucocerebroside or glucosylsphingosine, caused by glucocerebrosidase deficiency, may mediate brain inflammation in the Gaucher mouse via the elevation of proinflammatory cytokines, nitric oxide, and reactive oxygen species.
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의과대학 > 의학과 > Journal papers
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