Mäki T, Somer H, Pihko H, Lindlöf M, Kontula K, Härkönen M
Department of Clinical Chemistry, Children's Hospital, Helsinki, Finland.
J Neurol Sci. 1989 Sep;92(2-3):281-90. doi: 10.1016/0022-510x(89)90143-3.
Lymphocytic beta-adrenoceptor levels, receptor binding affinity, lymphocytic basal and isoproterenol-stimulated cyclic AMP (cAMP) production and plasma catecholamine levels were studied in 49 patients with neuromuscular diseases and in 10 healthy subjects. Patients with X-linked muscular dystrophy (Duchenne, 13 patients; Becker, 4 patients) showed a significant reduction in lymphocytic beta-adrenoceptor densities (35.9 +/- 2.2 fmol/mg protein vs. 49.6 +/- 3.6 fmol/mg, controls; P less than 0.02), whereas the receptor levels for the patients with spinal muscular atrophy (15), polymyositis (10) and for Duchenne carriers (7) did not differ significantly from the corresponding levels for the control subjects. The reduction in the beta-adrenoceptor density was not correlated with the physical disability of the patients. Lymphocytic beta-adrenoceptor affinity (KD) and basal and isoproterenol-stimulated cAMP levels were all comparable to control subjects' values. Catecholamine levels showed mild inconsistent elevations in various patient groups. The results suggest that reduction in cellular beta-adrenoceptors is characteristic of X-linked muscular dystrophies. Its relationship to the basic gene defect is unknown.
对49例神经肌肉疾病患者和10名健康受试者的淋巴细胞β - 肾上腺素能受体水平、受体结合亲和力、淋巴细胞基础及异丙肾上腺素刺激的环磷酸腺苷（cAMP）生成以及血浆儿茶酚胺水平进行了研究。患有X连锁肌营养不良症（杜氏，13例；贝克氏，4例）的患者淋巴细胞β - 肾上腺素能受体密度显著降低（35.9±2.2 fmol/mg蛋白质，对照组为49.6±3.6 fmol/mg；P<0.02），而脊髓性肌萎缩症患者（15例）、多发性肌炎患者（10例）及杜氏携带者（7例）的受体水平与相应对照组无显著差异。β - 肾上腺素能受体密度的降低与患者的身体残疾程度无关。淋巴细胞β - 肾上腺素能受体亲和力（KD）以及基础和异丙肾上腺素刺激的cAMP水平均与对照组相当。不同患者组的儿茶酚胺水平有轻度的不一致升高。结果表明，细胞β - 肾上腺素能受体减少是X连锁肌营养不良症的特征。其与基本基因缺陷的关系尚不清楚。
