Comim Clarissa M, Hoepers Andreza, Ventura Letícia, Freiberger Viviane, Dominguini Diogo, Mina Francielle, Mendonça Bruna P, Scaini Giselli, Vainzof Mariz, Streck Emílio L, Quevedo João
Laboratory of Experimental Neuroscience, Postgraduate Program in Health Sciences, University of Southern Santa Catarina, Palhoça, Santa Catarina, Brazil.
Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit, University of Southern Santa Catarina, Criciúma, Santa Catarina, Brazil.
Muscle Nerve. 2016 Jan;53(1):91-5. doi: 10.1002/mus.24704.
Duchenne muscular dystrophy (DMD) is a degenerative disease of skeletal, respiratory, and cardiac muscles caused by defects in the dystrophin gene. More recently, brain involvement has been verified. Mitochondrial dysfunction and oxidative stress may underlie the pathophysiology of DMD. In this study we evaluate Krebs cycle enzymes activity in the cerebral cortex, diaphragm, and quadriceps muscles of mdx mice.
Cortex, diaphragm, and quadriceps tissues from male dystrophic mdx and control mice were used.
We observed increased malate dehydrogenase activity in the cortex; increased malate dehydrogenase and succinate dehydrogenase activities in the diaphragm; and increased citrate synthase, isocitrate dehydrogenase, and malate dehydrogenase activities in the quadriceps of mdx mice.
This study showed increased activity of Krebs cycle enzymes in cortex, quadriceps, and diaphragm in mdx mice.
杜氏肌营养不良症（DMD）是一种由肌营养不良蛋白基因缺陷引起的骨骼肌、呼吸肌和心肌退行性疾病。最近，已证实脑部也会受到影响。线粒体功能障碍和氧化应激可能是DMD病理生理学的基础。在本研究中，我们评估了mdx小鼠大脑皮层、膈肌和股四头肌中 Krebs 循环酶的活性。
使用雄性营养不良mdx小鼠和对照小鼠的大脑皮层、膈肌和股四头肌组织。
我们观察到mdx小鼠大脑皮层中苹果酸脱氢酶活性增加；膈肌中苹果酸脱氢酶和琥珀酸脱氢酶活性增加；股四头肌中柠檬酸合酶、异柠檬酸脱氢酶和苹果酸脱氢酶活性增加。
本研究表明mdx小鼠大脑皮层、股四头肌和膈肌中 Krebs 循环酶的活性增加。
