Budget Amount *help |
¥20,540,000 (Direct Cost: ¥15,800,000、Indirect Cost: ¥4,740,000)
Fiscal Year 2013: ¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
Fiscal Year 2012: ¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2011: ¥13,260,000 (Direct Cost: ¥10,200,000、Indirect Cost: ¥3,060,000)
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Research Abstract |
By analyzing GPHR-deficient mouse embryonic fibroblast (MEF) in which major pathology is a raised Golgi pH, we clarified 1) impaired glycosylation is caused by reduced activities of nucleotide-sugar transporter and aberrant complex formation of glycosyltransferases but not by mislocalization of glycosyltransferases, 2) both transcriptions of several genes involved in cholesterol biosynthesis and the amount of intracellular cholesterol are reduced in GPHR-deficient MEF and it is most likely that the phenotype is caused by abnormal cholesterol distribution due to the impaired cholesterol trafficking through the membrane contact site between the ER and Golgi apparatus, and 3) skin basal cell-specific gene targeting revealed that GPHR is essential for the homeostasis of the epidermis including the formation of lamellar bodies and for the barrier function.
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