2014 Fiscal Year Final Research Report
Analysis of peroxisomal disorders from the structure of GPI anchors
Project/Area Number |
25860238
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Research Category |
Grant-in-Aid for Young Scientists (B)
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Allocation Type | Multi-year Fund |
Research Field |
Pathological medical chemistry
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Research Institution | Osaka University |
Principal Investigator |
KANZAWA Noriyuki 大阪大学, 微生物病研究所, 特任助教(常勤) (40452461)
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Project Period (FY) |
2013-04-01 – 2015-03-31
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Keywords | ペルオキシソーム病 / グリコシルホスファチジルアンカー / アルキル脂質 / 脂質リモデリング |
Outline of Final Research Achievements |
Many proteins are anchored by a glycosylphosphatidylinositol (GPI) to the cell surface. GPI is synthesized from phosphatidylinositol (PI). Most PIs are diacyl form, whereas majority of mammalian GPI-anchored proteins (GPI-APs) have alkyl-acyl PIs. I determined that peroxisomal pathway is essential for production of alkyl-acyl form GPIs. I predicted that peroxisomal pathway generate alkyl donor of lipid remodeling, and I tried to identify the remodelase and analyze mechanisms. A defect in the alkyl-phospholipid biosynthesis causes peroxisomal disorders, and these are lethal genetic diseases. These patients are defective in alkyl-acyl form GPIs, and absence of the alkyl-acyl form of GPI-APs might account for some of the complex phenotypes of peroxisomal disorders. Although functional importance of the alkyl-acyl form of GPI-APs is yet to be determined, I am currently testing the possibility that the lack of alkyl-acyl form GPI-APs contributes to some of the symptoms.
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Free Research Field |
分子病態学
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