Role of Carnitine on Lipid and Carbohydrate Metagolism

肉碱对脂质和碳水化合物代谢的作用

• 批准号: 04671476
• 负责人: KUWAJIMA Masamichi
• 金额: $ 1.34万
• 依托单位: Osaka University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (C)
• 财政年份: 1992
• 资助国家: 日本
• 起止时间: 1992 至 1993
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-04671476/
• 关键词: carnitine; cardiomegaly; mitochondria; long chain fatty acid; mit DNA; fatty liver; heart failure; carnitine transport activity; 総脂質; トリアシルグリセロール; 高アンモニア血症; 高脂血症; 尿素サイクル

To evaluate the role of carnitine on lipid and carbohydrate metabolism, we analyzed the metabolic disorders in congenital systemic carnitine deficient mouse (jvs mouse). Results are as follows ;1. In an experiment to assay the uptake of carnitine using kidney slice, homozygous mutants showed significantly lower rates of Na-dependent carnitine uptake than controls. This results indicate that the primary defect of jvs mice is most probably related to the system for reabsorption of carnitine in the kidney.2. Jvs mice show cardiac hypertrophy which is caused by enlargement of the cardiac muscle cell associated with enlargement of mitochondria. Carnitine administration significantly suppresses the cardiac hypertrophy. These results show that carnitine deficiency plays an important role in the development of the cardiac hypertrophy.

为探讨肉毒碱对脂代谢和糖代谢的影响，我们对先天性系统性肉毒碱缺乏小鼠（jvs小鼠）的代谢紊乱进行了研究。结果如下：1.在使用肾切片测定肉毒碱摄取的实验中，纯合突变体显示出比对照显著更低的Na依赖性肉毒碱摄取速率。这一结果表明jvs小鼠的原发性缺陷很可能与肾脏对肉毒碱的重吸收系统有关. Jvs小鼠表现出心肌肥大，这是由与线粒体增大相关的心肌细胞增大引起的。肉毒碱管理显着抑制心肌肥厚。结果表明，肉毒碱缺乏在心肌肥厚的发生发展中起重要作用。

项目成果

V.Esser,M.Kuwajima,C.H.Britton,K.Krishnan,J.D.McGarry: "Inhibitors of mitochondrial carnitine palmitoyltransferase I limit the action of proteases on the enzyme" J.Biol.Chem.268. 5810-5816 (1993)

V.Esser、M.Kuwajima、C.H.Britton、K.Krishnan、J.D.McGarry：“线粒体肉碱棕榈酰转移酶 I 的抑制剂限制蛋白酶对该酶的作用”J.Biol.Chem.268。

M.Horiuchi, K.Kobayashi, M.Tomomura, M.Kuwajima, Y.Imamura, T.Koizumi, H.Nikaido, J.Hayakawa, T.Saheki: "Carnitine administration to juvenile visceral steatosis (jvs) mouse corrects the suppressed expression of the urea cycle enzyme genes" J.Biol. Chem.26

M.Horiuchi、K.Kobayashi、M.Tomomura、M.Kuwajima、Y.Imamura、T.Koizumi、H.Nikaido、J.Hayakawa、T.Saheki：“对幼年内脏脂肪变性 (jvs) 小鼠施用肉碱可纠正抑制的内脏脂肪变性

V.Esser et al.: "Inhibitors of mitochondrial carnitine palmitoyltranoferare I limit the action of proteases on the enzyme." J.Biol.Chem.268. 5810-5816 (1993)

V.Esser 等人：“线粒体肉毒碱棕榈酰转移酶抑制剂 I 限制了蛋白酶对该酶的作用。”

T.Nishimura: "Influence of daily drinking habits on ethanal-induced hyperuricemia." Metabolism. (印刷中).

T. Nishimura：“日常饮酒习惯对乙醛引起的高尿酸血症的影响”（正在出版）。

H.Nakajima: "An alternative,non-isotopic protocol of PCR-mediated allele-specific oligonucleotide analysis for the detection of a point mutation" Res.Commun.Chem.Pathol.Pharmacol.79. 3-10 (1993)

H.Nakajima：“用于检测点突变的 PCR 介导的等位基因特异性寡核苷酸分析的替代非同位素方案”Res.Commun.Chem.Pathol.Pharmacol.79。