Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis.
Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis.
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囊性纤维化患者淋巴细胞和粒细胞中的 β 肾上腺素受体。
DOI：
10.1172/jci110934
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Doershuk,CF
中科院分区：
文献类型：
--
作者：
Davis,PB;Dieckman,L;Boat,TF;Stern,RC;Doershuk,CF
囊性纤维化（CF）患者的完整淋巴细胞对异丙肾上腺素（10（-8）-10（-4）M）的反应产生的环磷酸腺苷（cAMP）显著低于正常淋巴细胞（P <0.001），尽管基础cAMP含量和对前列腺素E1的反应正常。CF的专性杂合子也显著（P <0.005）降低了cAMP对异丙肾上腺素的反应，表明CF中β肾上腺素能缺乏的遗传成分。通过[3 H]二氢阿普洛尔与淋巴细胞颗粒的平衡结合测定的β肾上腺素能受体数量在正常淋巴细胞（969 +/- 165受体/细胞）和CF患者淋巴细胞（1，333 +/- 263受体/细胞）中相同。通过二氢烯丙洛尔的KD和（-）-异丙肾上腺素的Ki评估，拮抗剂和激动剂受体的结合特性在CF淋巴细胞中也正常。类似地，与健康对照组相比，CF患者的粒细胞对异丙肾上腺素（10（-8）-10（-4）M）的cAMP反应显著降低（P <0.03），专性杂合子的粒细胞也是如此（P <0.05）。同样，基础cAMP水平和对前列腺素E1的反应是正常的。通过[3 H]二氢阿普洛尔与粒细胞颗粒的平衡结合测定的β肾上腺素能受体数量在正常（1，462 +/- 249受体/细胞）和CF（1，621 +/- 221受体/细胞）制剂中相同。通过二氢阿普洛尔的KD和异丙肾上腺素的Ki评估的激动剂和拮抗剂受体的结合特性在CF粒细胞颗粒中正常。CF中的淋巴细胞和粒细胞β肾上腺素能缺陷不能用β肾上腺素能受体或腺苷酸环化酶本身的异常来解释。受体环化酶偶联是CF中遗传性β肾上腺素能缺陷的最可能位点。
Intact lymphocytes from patients with cystic fibrosis (CF) produce significantly (P less than 0.001) less adenosine 3':5' cyclic monophosphate (cAMP) than normal lymphocytes in response to isoproterenol (10(-8)-10(-4) M), although the basal cAMP content and the response to prostaglandin E1 are normal. Obligate heterozygotes for CF have significantly (P less than 0.005) reduced cAMP response to isoproterenol as well, suggesting a genetic component in the beta adrenergic deficiency in CF. The number of beta adrenergic receptors, as determined by equilibrium binding of [3H]dihydroalprenolol to lymphocyte particulates, is the same in normal lymphocytes (969 +/- 165 receptors/cell) and lymphocytes from patients with CF (1,333 +/- 263 receptors/cell). Binding properties of the receptor for both antagonist and agonist, as assessed by KD for dihydroalprenolol and Ki for (-)-isoproterenol, are also normal in the CF lymphocytes. Similarly, in granulocytes from patients with CF, the cAMP response to isoproterenol (10(-8)-10(-4) M) is significantly reduced compared with healthy controls (P less than 0.03), as is the response of granulocytes from obligate heterozygotes (P less than 0.05). Again, the basal cAMP levels and the response to prostaglandin E1 are normal. The number of beta adrenergic receptors, as determined by equilibrium binding of [3H]dihydroalprenolol to granulocyte particulates, was the same in normal (1,462 +/- 249 receptors/cell) and CF (1,621 +/- 221 receptors/cell) preparations. Binding properties of the receptor for both agonist and antagonist, as assessed by KD for dihydroalprenolol and Ki for isoproterenol, are normal in CF granulocyte particulates. The lymphocyte and granulocyte beta adrenergic defect in CF cannot be explained by abnormalities of the beta adrenergic receptor or of adenylate cyclase itself. Receptor-cyclase coupling is the most likely site of the heritable beta adrenergic defect in CF.
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影响因子：
3.3
作者：
C. Doershuk;L. Matthews;A. Tucker;Harry Nudelman;George Eddy;M. B. Wise;S. Spector
通讯作者：
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DOI：
10.1097/00006254-198102000-00017
发表时间：
1980
期刊：
The New England journal of medicine
影响因子：
--
作者：
Z. Farfel;A. Brickman;H. Kaslow;H. Bourne
通讯作者：
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DOI：
10.1164/arrd.1981.123.6.622
发表时间：
1981
期刊：
The American review of respiratory disease
影响因子：
--
作者：
LemanskeJr,RF;Mischler,EH;Farrell,PM;Anderson,C;Busse,WW
通讯作者：
Busse,WW
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DOI：
--
发表时间：
1976
期刊：
Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie
影响因子：
--
作者：
C. Arkesteijn
通讯作者：
C. Arkesteijn
囊性纤维化中肾上腺素能和胆碱能敏感性异常。
影响因子：
158.5
作者：
P. Davis;J. Shelhamer;M. Kaliner
通讯作者：
M. Kaliner