Biochemical study on lipid metabolism in lipid storage myopathy
脂质沉积性肌病脂质代谢的生化研究
基本信息
- 批准号:62570366
- 负责人:
- 金额:$ 1.22万
- 依托单位:
- 依托单位国家:日本
- 项目类别:Grant-in-Aid for General Scientific Research (C)
- 财政年份:1987
- 资助国家:日本
- 起止时间:1987 至 1988
- 项目状态:已结题
- 来源:
- 关键词:
项目摘要
We studied lipid metabolism in clutured fibroblasts from a patient with lipid storage myopathy. The patient has slowly progressive myopathy, pigmentary retinal degeneration, neurosensory hearing loss. There were many vacuoles containing neutral lipid in skeletal muscle fibers, leukocytes, and cultured fibroblasts.Carnitine content and the activities of carnitine palmitoyltransferase and mitochondrial electron transport system were normal, and the ratio of acylcarnitine to free carnitine was elevated in skeletal muscle. The level of triglyceride content of the cultured fibroblasts was ten times higher than that of controls, and fatty acid composition was normal. The oxidation of long-chain fatty acid was impaired (37% of control values), while the oxidation reaction of medium-chain and short-chain fatty acid were normal in the cultured fibroblasts. The activities of enzymes working on long-chain fatty acid oxidation were normal. When the fibroblasts were fed with radiolabeled oleate for 6 hrs, the triglyceride synthsis was increased twice as controls. Pulse-chase experiment showed the reduced degradation of synthesized triglyceride. The activities of acidic, neutral, and alkaline lipase were normal level in fibroblasts extracts.The regulation in both long-chain fatty acid oxidation and triglyceride degradation may be impaired, which causes the intracellure accumulation.
我们研究了从一个患有脂质沉积性肌病的患者培养的成纤维细胞中的脂质代谢。患者有缓慢进行性肌病、色素性视网膜变性、感觉神经性听力丧失。骨骼肌纤维、白细胞和培养的成纤维细胞中有许多含中性脂质的空泡,骨骼肌中肉毒碱含量、肉毒碱棕榈酰转移酶和线粒体电子传递系统活性正常,酰基肉毒碱/游离肉毒碱比值升高。培养的成纤维细胞的甘油三酯含量水平比对照组高10倍,脂肪酸组成正常。长链脂肪酸的氧化受损(对照值的37%),而中链和短链脂肪酸的氧化反应在培养的成纤维细胞中是正常的。长链脂肪酸氧化酶活性正常。当用放射性标记的油酸盐喂养成纤维细胞6小时时,甘油三酯增加两倍于对照。脉冲追踪实验表明,合成的甘油三酯的降解减少。成纤维细胞提取液中酸性、中性和碱性脂肪酶活性均正常,长链脂肪酸氧化和甘油三酯降解的调节可能受损,导致细胞内蓄积。
项目成果
期刊论文数量(3)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
Fumihisa Soga: "Lipids in muscle cell" Journal of Clinical Science. 24(4). 515-520 (1988)
Fumihisa Soga:“肌肉细胞中的脂质”临床科学杂志。
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UENO Satoshi其他文献
Trends in health information and communication standards in Japan
日本健康信息和通信标准的趋势
- DOI:
10.20683/jniph.69.1_52 - 发表时间:
2020 - 期刊:
- 影响因子:0
- 作者:
KIMURA Eizen;UENO Satoshi - 通讯作者:
UENO Satoshi
UENO Satoshi的其他文献
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{{ truncateString('UENO Satoshi', 18)}}的其他基金
Catalytic Addition of be-ta-C-H Bond in Aliphatic Carbonyl Derivatives to Unsaturated Bonds
脂肪族羰基衍生物中β-C-H键与不饱和键的催化加成
- 批准号:
16K21441 - 财政年份:2016
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Young Scientists (B)
Repairing DNA damages in neurodegenerative diseases
修复神经退行性疾病中的 DNA 损伤
- 批准号:
20591008 - 财政年份:2008
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Development of an Axial Self-bearing Motor/Generator
轴向自轴承电动机/发电机的开发
- 批准号:
19760181 - 财政年份:2007
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Young Scientists (B)
Analyses for the function and related gene of humanin that suppresses cell death induced by mutant Alzheimer disease genes
护脑素抑制阿尔茨海默病突变基因诱导的细胞死亡的功能及相关基因分析
- 批准号:
14570612 - 财政年份:2002
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Molecular analysis of hereditary progressive dystonia
遗传性进行性肌张力障碍的分子分析
- 批准号:
10670597 - 财政年份:1998
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Gene analysis of the superoxide dismutase gene in amyotrophic lateral screlosis
肌萎缩侧索硬化超氧化物歧化酶基因的基因分析
- 批准号:
07670725 - 财政年份:1995
- 资助金额:
$ 1.22万 - 项目类别:
Grant-in-Aid for Scientific Research (C)