@article{oai:niigata-u.repo.nii.ac.jp:00021271, author = {小池, 亮子}, issue = {10}, journal = {新潟医学会雑誌, 新潟医学会雑誌}, month = {Oct}, note = {The metabolism of radioactive fatty acids in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and Zellweger syndrome (ZS) were studied to clarify the physiological significance of the fatty acid elongation system in the accumulation of very long chain fatty acids (VLFA) in ALD. A substantial amount of radioactive C26:0 was synthesized from [18-^<14>C] stearic acid by ALD and ZS fibroblasts, whereas radioactive C26:0 was not detected in control fibroblasts. Kinetic studies demonstrated that the production of radioactive C24:0 and C26:0 in ALD fibroblasts is higher than that of ZS fibroblasts, which suggests that the fatty acid elongation activity plays an important role for the accumulation of VLFA in ALD, in addition to the decreased VLFA oxidation activities. The addition of monounsaturated fatty acids including oleic acid and erucic acid specifically lowered the formation rate of VLFA without any significant effect on degradation activities of VLFA. both in ALD and control fibroblasts. The result suggest that the mechanism of decrease of VLFA by administration of monounstaurated fatty acids is based on the inhibition of fatty acid elongation activity.}, pages = {688--695}, title = {Adrenoleukodystrophyにおける脂肪酸鎖長延長活性の役割ならびに単価不飽和脂肪酸による治療効果のメカニズムについて}, volume = {105}, year = {1991} }