| Project/Area Number |
25860238
|
|---|
| Research Category |
Grant-in-Aid for Young Scientists (B)
|
| Allocation Type | Multi-year Fund |
|---|
| Research Field |
Pathological medical chemistry
|
|---|
| 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)
|
|---|
| Project Period (FY) |
2013-04-01 – 2015-03-31
|
| Project Status |
Completed (Fiscal Year 2014)
|
| Budget Amount *help |
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2013: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
|
|---|
| 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 / 先天性代謝異常 / GPIアンカー |
|---|
| 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.
|
