1986 Fiscal Year Final Research Report Summary
Study of the pathogenesis of adrenoleukodystrophy
| Project/Area Number |
60480220
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | Niigata University |
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Principal Investigator |
MIYATAKE Tadashi Brain Research Institute, Niigata University ・ Professor, 脳研究所, 教授 (50048998)
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| Co-Investigator(Kenkyū-buntansha) |
SATOU Syuzou University Hospital, Niigata University ・ Assistant, 医学部付属病院, 講師 (30115034)
ATUMI Tetushi Brain Research Institute, Niigata University ・ Assistant professor, 脳研究所, 助教授 (50049061)
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| Project Period (FY) |
1985 – 1986
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| Keywords | Adrenoleukodystrophy / Zellwerger Syndrome / Zellweger症候群 |
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| Research Abstract |
We have studied the metabolism of long chain fatty acids in cultured skin fibroblasts from patients with adrenoleukodystrophy(ALD). The composition of radioactive fatty acids in total lipids of fibroblasts after culture in the presence of <^3H_2> -hexacosanoic acid on <^3H> -lignoreric acid was analyzed by radio-gaschromatography. Most of the fatty acids added to the culture media were degraded to shorter-chain fatty acids both by control and ALD fibroblasts. However, the content of the remaining radioactive <C_(24:0)> or <C_(26:0)> was higher in ALD fibroblast. This result indicated that the degradation of very long chain fatty acids (VLCFA) is decreased in ALD.
Next, we examined the VLCFA metabolism in fibroblasts from Zellweger syndrome. The increased synthesis of <C_(26:0)> from <C_(18:0)> was found in the fibroblasts of Zellweger syndrome as well as ALD.
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