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Downregulation of neurotrophic factors in the brain of a mouse model of Gaucher disease; implications for neuronal loss in Gaucher disease

Title
Downregulation of neurotrophic factors in the brain of a mouse model of Gaucher disease; implications for neuronal loss in Gaucher disease
Authors
Kim, Eun YoungHong, Young BinGo, Sang HeeLee, BeobyiJung, Sung-Chul
Ewha Authors
정성철
SCOPUS Author ID
정성철scopus
Issue Date
2006
Journal Title
EXPERIMENTAL AND MOLECULAR MEDICINE
ISSN
1226-3613JCR Link
Citation
vol. 38, no. 4, pp. 348 - 356
Keywords
brainbrain-derived neurotrophic factorGaucher diseaseglucosylceramidasenerve growth factorneurons
Publisher
KOREAN SOC MED BIOCHEMISTRY MOLECULAR BIOLOGY
Indexed
SCI; SCIE; SCOPUS; KCI WOS scopus
Abstract
Gaucher disease is a glycosphingolipid storage disease caused by deficiency of glucocerebrosidase, resulting in the accumulation of glucosylceramide in lysosomes. The neuronopathic forms of this disease are associated with neuronal loss and neurodegeneration. However, the pathophysiological mechanisms leading to prenatal and neonatal death remain uncharacterized. To investigate brain dysfunction in Gaucher disease, we studied the effects of neurotrophic factors during development in a mouse model of Gaucher disease. The expression of brain-derived neurotrophic factor and nerve growth factor was reduced in the cerebral cortex, brainstem, and cerebellum of Gaucher mice, compared with that in wild-type mice. Extracellular signal-regulated kinase (ERK) 1/2 expression was downregulated in neurons from Gaucher mice and correlated with a decreased number of neurons. These results suggest that a reduction in neurotrophic factors could be involved in neuronal loss in Gaucher disease.
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의학전문대학원 > 의학과 > Journal papers
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