| Project/Area Number |
09671435
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Cerebral neurosurgery
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| Research Institution | Yokohama City University |
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Principal Investigator |
KANNO Hiroshi Yokohama City Uni., Medicine, Assist.Prof, 医学部, 講師 (40244496)
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| Co-Investigator(Kenkyū-buntansha) |
YAMAMOTO Isao Yokohama City Uni., Medicine, Prof., 医学部, 教授 (30158266)
KAWAMOTO Susumu Yokohama City Uni., Medicine, Assist.Prof, 医学部, 講師 (80125921)
HATTORI Satoshi Yokohama City Uni., Medicine, Instructer, 医学部, 助手 (40275037)
NAGASHIMA Yoji Yokohama City Uni., Medicine, Assist.Prof, 医学部, 講師 (10217995)
YAO Masahiro Yokohama City Uni., Medicine, Assist.Prof., 医学部, 講師 (00260787)
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| Project Period (FY) |
1997 – 1998
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| Project Status |
Completed (Fiscal Year 1998)
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| Budget Amount *help |
¥2,900,000 (Direct Cost: ¥2,900,000)
Fiscal Year 1998: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 1997: ¥1,900,000 (Direct Cost: ¥1,900,000)
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| Keywords | von Hippel-Lindau disease / Central nervous system / Hemangioblastoma / DNA diagnosis / Neuronal differentiation / クォン・ヒッペル・リンド-病 / 腫瘍抑制遺伝子 / 母斑病 / 遺伝性脳腫瘍 |
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| Research Abstract |
Von Hippel-Lindau disease (VHL) is an autosomal dominant hereditary disease which develops hamangioblastomas in the central nervous system and retina, renal cell carcinoma, and pheochromocytoma. The causative gene has been isolated from chromosome 3p in 1993. We have found somatic VHL gene mutations in not only sporadic hemangioblastomas and renal cell but also gliomas. In addition, we have performed molecular genetic diagnosis of 80 Japanese VHL families by the DNA analyses. The expressions of VHL gene and product were identified at neuron in the central nervous system and proximal renal tubule as well as cytoplasm of hemangiobalstoma and glioma. However, pathologically more malignant gliomas showed less expression of VHL gene produst. In process of neuronal development, VHL gene expressions were correlated with neuronal differentiation in neural stem cells. In addtion, it was suggested that cell cycle of neural stem cells were down-regulated by VHL gene.
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