Khokhlov A P, Malakhovskiĭ V K
Vopr Med Khim. 1978 Nov-Dec;24(6):754-8.
Concentration of cyclic AMP was distinctly decreased in muscular tissue of patients with Dushenn's myodystrophy. Simultaneously, in patients with the myopathy basal activity of adenylate cyclase as well as activation of the enzyme by adrenaline and sodium fluoride were decreased. As a compensation, activity of phosphodiesterase was also decreased. Content of cAMP, activity and activation of adenylate cyclase were similar to normal state in patients with Erb's and Landuzi--Dejerin's myopathies; activity of phosphodiesterase was increased in the latter impairment. Dissociation constant for binding of cAMP by protein kinases was more than 10-fold decreased in patients with these types of myopathy.
杜兴氏肌营养不良症患者肌肉组织中环状AMP的浓度明显降低。同时,患有肌病的患者中,腺苷酸环化酶的基础活性以及肾上腺素和氟化钠对该酶的激活作用均降低。作为一种补偿,磷酸二酯酶的活性也降低。在埃布氏和兰杜齐 - 德热里纳氏肌病患者中,cAMP的含量、腺苷酸环化酶的活性及激活情况与正常状态相似;在后者的损伤中,磷酸二酯酶的活性增加。这些类型肌病患者中,蛋白激酶结合cAMP的解离常数降低了10倍以上。