1988 Fiscal Year Final Research Report Summary
Studies on pathogenesis of Zellweger syndrome
| Project/Area Number |
61304046
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| Research Category |
Grant-in-Aid for Co-operative Research (A)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | TOHOKU UNIVERSITY |
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Principal Investigator |
TADA Keiya Department of Pediatrics, Tohoku University School of Medicine, 医学部, 教授 (20046907)
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| Co-Investigator(Kenkyū-buntansha) |
AIKAWA Junitiro Department of Pediatrics, Tohoku University School of Medicine, 助手 (30191844)
YOKOTA Sadaki Department of Anatomy, Yamanashi Medical College, 助教授 (40020755)
ORII Tadao Department of Pediatrics, Gifu University School of Medicine, 医学部, 教授 (20045339)
HASHIMOTO Takashi Department of Biochemistry, Shinshu University Medical School, 医学部, 教授 (80009935)
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| Project Period (FY) |
1986 – 1988
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| Keywords | Zellweger syndrome / Peroxisomes / beta-oxidation / β酸化系酵素 |
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| Research Abstract |
This study was undertaken in order to elucidate pathogenesis of Zellweger syndrome (ZS), in which peroxisomes do not exist in cells. The following results were obtained 1) The defect of enzymes involved in peroxisomal -oxidation was recognized in cells from the patients with ZS. 2) The defect of peroxisomal membrane proteins of 70 KD, 26 KD and 22 KD was found in cells from the patient with ZD. 3) The enzymes involved in mitochondrial -oxidation were all intact in ZS cells. 4) It was suggested that the peroxisomal enzymes can be synthesized in ZS cells, but they are rapidly decomposed in cytoplasma because of the absence of peroxisomes.
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