2011 Fiscal Year Final Research Report
Proteoglycan quality control mechanisms by the tumor suppressor gene EXTL2
| Project/Area Number |
22659019
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Single-year Grants |
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| Research Field |
Biological pharmacy
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| Research Institution | Kobe Pharmaceutical University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
NADANAKA Satomi 神戸薬科大学, 薬学部, 講師 (60378578)
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| Project Period (FY) |
2010 – 2011
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| Keywords | プロテオグリカン / グリコサミノグリカン / 糖転移酵素 / ガン抑制遺伝子 / コンドロイチン硫酸 / へパラン硫酸 / 遺伝性多発性外骨腫 / キナーゼ |
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| Research Abstract |
Heparan sulfate(HS) is synthesized by HS co-polymerases encoded by the EXT1and EXT2genes, which are known as causative genes for hereditary multiple exostoses, a dominantly inherited genetic disorder characterized by multiple cartilaginous tumors. Three EXT-like(EXTL) genes that share significant sequence homologies with EXT1and EXT2have been identified in mammals. However, their roles in HS biosynthesis remain unclear. Here, we showed that the transfer of the first GlcNAc residue to the linkage region by EXTL2is critically required for the biosynthesis of HS in cells deficient in EXT1and is likely involved in the quality control mechanism of proteoglycans.
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