FATTY ACID METABOLISM IN DYSTROPHIC MUSCLE IN VITRO
FATTY ACID METABOLISM IN DYSTROPHIC MUSCLE IN VITRO
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DOI：
10.1016/0024-3205(69)90112-x
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发表时间：
1969-01-01
期刊：
影响因子：
--
通讯作者：
STRICKLAND, KP
中科院分区：
文献类型：
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作者：
LIN, CH;HUDSON, AJ;STRICKLAND, KP
最近对营养不良肌肉中线粒体的生化研究揭示了代谢缺陷，这无疑是肌病过程中的一个主要因素。已经描述了一种类型的骨骼肌疾病，其中线粒体显示出氧化磷酸化的松散偶联状态（1，2），科尔曼等人。(3)已经描述了一种具有线粒体酶活性过高的肌病。在后一种情况下，存在脂肪的异常积累，作者推测这是由于线粒体氧化脂肪的能力受损。在某些类型的肌营养不良症中，骨骼肌中脂质的异常增加是众所周知的，并且线粒体可能在其积累中起重要作用。由于营养不良小鼠的骨骼肌中存在过量脂质，因此对肌肉匀浆中的脂肪酸氧化以及肌肉线粒体中的脂肪酸和脂肪酸氧化进行了研究，以确定是否存在缺陷。脂质的量还取决于其形成的速率，因此还研究了脂肪酸的合成。材料和方法将营养不良小鼠（品系129）和它们的同窝对照（年龄60-98天）的两种性别的小鼠断头，迅速取出后腿肌肉，称重并悬浮在含有1 mM EDTA的冰冷的0.25 M蔗糖中。将肌肉切成小块，并在宽松的全玻璃Potter-Elvejehm匀浆器中轻轻匀浆60秒。在紧密配合的均质器中再进行均质化两分钟。的
Recent biochemical studies of mitochondria in dystrophic muscle have revealed metabolic defects that are undoubtedly a major factor in the myopathic process. A type of skeletal muscle disease has been described in which the mitochondria showed a loosely coupled state of oxidative phosphorylation (1, 2) and Coleman et al.(3) have described a type of myopathy with mitochondrial enzyme hyperactivity. In the latter case, there was an abnormal accumulation of fat that the authors postulated was due to an impaired capacity of mitochondria to oxidize fat. An abnormal increase of lipid in the skeletal muscle in some types of muscular dystrophy is well known and it is probable that mitochondria have an important role in its accumulation. Since excess lipid is present in the skeletal muscle of dystrophic mice, a study was made of fatty acid oxidation in muscle homogenate and of pyruvic and fatty acid oxidation in muscle mitochondria to determine whether a defect exists. The amount of lipid is also dependent upon the rate at which it is formed and therefore fatty acid synthesis was also investigated.: Saterials and Methods Dystrophic mice (Strain 129) and their littermate controls (ages 60-98 days) of both sexes were decapitated and the hind leg muscles were rapidly removed, weighed and suspended in ice-cold 0.25 M sucrose containing 1 mM EDTA. The muscle was cut into small pieces and homogenized gently in a loose-fitting all glass Potter-Elvejehm homogenizer for 60 seconds. Homogenization was carried out another two minutes in a tightly fitting homogenizer. The