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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2006.1
- 수록면
- 348 - 356 (9page)
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disease caused by deficiency of glucocerebro-sidase, resulting in the accumulation of glu-cosylceramide in lysosomes. The neuronopathic forms of this disease are associated with neuronal loss and neurodegeneration. However, the patho-physiological mechanisms leading to prenatal and neonatal death remain uncharacterized. To in-vestigate 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. trophic factors could be involved in neuronal loss in Gaucher disease.
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참고문헌 (29)
참고문헌 신청
/ / A branched signaling pathway for nerve growth factor is revealed by Src- : 3146 ~ 55
/ / Metabolism of glucocere-brosidase Evidence of an enzymatic deficiency in Gauchers disease : 221 ~ 5
/ / Mice lacking nerve growth factor display peri-natal loss of sensory and sympathetic neurons yet develop basal forebrain cholinergic neurons : 1001 ~ 11
/ / Targeted dis-ruption of the BDNF gene perturbs brain and sensory neuron development but not motor neuron development : 989 ~ 99
/ / The role of neuro-genetics in Gaucher disease : 1212 ~ 24
/ / Metabolism of glucocere-brosidase Evidence of an enzymatic deficiency in Gauchers disease : 221 ~ 5
/ / Mice lacking nerve growth factor display peri-natal loss of sensory and sympathetic neurons yet develop basal forebrain cholinergic neurons : 1001 ~ 11
/ / Targeted dis-ruption of the BDNF gene perturbs brain and sensory neuron development but not motor neuron development : 989 ~ 99
/ / The role of neuro-genetics in Gaucher disease : 1212 ~ 24
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