Hereditary Multiple Exostoses : Association with EXT Family Genes and Defective Heparan Sulfate Biosynthesis
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概要
- 論文の詳細を見る
Hereditary multiple exostoses, (HME) are benign bone tumors genetically associated with mutations in either EXTJ or EXT2, both of which have tumor suppressor functions. Subsequent cloning of three EXT-like genes, EXTL1, EXTL2 and EXTL3, has revealed the EXT gene family, which has been evolutionally conserved in Drosophila melanogaster and Caenorhabditis elegans. Recent studies identified EXT1 and EXT2 as heparan sulfate (HS) polymerases, which revealed the etiological connection between the HS biosynthesis and HME. Identification of GlcNAc transferase activities for the EXT-like proteins has implicated all EXT family members in the HS production. Several lines of accumulating evidence suggest that HS is indispensable to signalings critically involved in the regulation of bone growth, and that aberrant KS synthesis appears to explain the development of HME in humans.
- 日本結合組織学会の論文
- 2002-09-25
著者
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KITAGAWA Hiroshi
Department of Bioresource Science, Graduate School of Agricultural Science, Kobe University
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SUGAHARA Kazuyuki
Department of Laboratory Medicine, Nagasaki University Graduate School of Biomedical Sciences
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Sugahara Kazuyuki
Department Of Biochemistry Kobe Pharmaceutical University
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Sugahara K
Department Of Biochemistry Kobe Pharmaceutical University
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Kim Byung-taek
Department Of Biochemistry Kobe Pharmaceutical University
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Kitagawa Hiroshi
Department Of Biochemistry Kobe Pharmaceutical University
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Kitagawa Hiroshi
Department Of Adaptive Machine Systems Graduate School Of Engineering Osaka University
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