Oncogenic osteomalacia: evidence for a humoral phosphaturic factor.

Oncogenic osteomalacia: evidence for a humoral phosphaturic factor.
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致癌性骨软化症:体液磷酸盐因子的证据。

DOI:
10.1210/jcem.80.5.7745010
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发表时间:
1995
期刊:
The Journal of clinical endocrinology and metabolism
影响因子:
--
通讯作者:
G. Bondy
G. Bondy
中科院分区:
--
文献类型:
--
作者:
G. Wilkins;S. Granleese;R. Hegele;J. Holden;D. Anderson;G. Bondy

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瘤源性骨软化症是一种以磷酸盐尿、低磷酸盐血症、维生素D水平降低和骨软化为特征的综合征。病因尚不清楚,但所有患者都有肿瘤;通常是间叶起源的。切除肿瘤可以逆转代谢异常。我们报告一个病人骨软化症,严重的低磷酸盐血症,碱性磷酸酶升高,低1,25-二羟维生素D3,磷酸盐尿。在颞下窝发现了一个肿瘤。肿瘤被切除,所有的生化异常在随后的8个月内解决。骨密度恢复正常。该肿瘤具有副神经节瘤的外观,并用于建立称为JH-55的细胞培养系。原始肿瘤和JH-55细胞的电子显微镜检查表明存在神经分泌颗粒。使用负鼠肾细胞的生物测定法用于评价磷酸盐转运。来自JH-55细胞的条件培养基抑制肾小管细胞对磷酸盐的重吸收。最大抑制需要24小时的孵育期,并没有改变蛋白质合成的抑制剂(10微克/毫升放线菌酮)的存在。免疫分析显示,在JH-55培养基中没有检测到PTH相关肽或完整的PTH。这种副肿瘤综合征的原因尚不清楚,但所有的证据都与产生磷酸尿的激素的作用一致。这种假定的因子与引起磷酸尿的其他激素不同。
Oncogenic osteomalacia is a syndrome characterized by phosphaturia, hypophosphatemia, reduced vitamin D levels, and osteomalacia. The cause is not known, but all patients have had a tumor; usually of mesenchymal origin. Removal of the tumor reverses the metabolic abnormalities. We report a patient with osteomalacia, severe hypophosphatemia, elevated alkaline phosphatase, low 1,25-dihydroxyvitamin D3, and phosphaturia. A tumor was identified in the infratemporal fossa. The tumor was removed, and all of the biochemical abnormalities resolved over the subsequent 8 months. The bone density returned to normal values. The tumor had the appearance of a paraganglioma and was used to establish a cell culture line called JH-55. Electron microscopy of the original tumor and the JH-55 cells demonstrated the presence of neurosecretory granules. A bioassay using opossum kidney cells was used to evaluate phosphate transport. Conditioned medium from the JH-55 cells inhibited phosphate reabsorption by the kidney tubular cells. Maximal inhibition required a 24-h incubation period and was not altered by the presence of an inhibitor of protein synthesis (10 micrograms/mL cycloheximide). Immunoassays revealed no detectable PTH-related peptide or intact PTH in the JH-55 medium. The cause of this paraneoplastic syndrome is not known, but all of the evidence is consistent with the action of a hormone that produces phosphaturia. This putative factor is distinct from other hormones that cause phosphaturia.
甲状旁腺激素相关蛋白:结构、功能和测量。
DOI: --
发表时间: 1992
期刊: Clinical chemistry
影响因子: 9.3
作者:
Burtis,WJ
通讯作者: Burtis,WJ
低磷血症 (Hyp) 小鼠肾皮质中钙和降钙素对 25-羟基维生素 D3-1 α-羟化酶活性的异常调节。
DOI: 10.1210/endo-114-4-1203
发表时间: 1984
期刊: Endocrinology
影响因子: 4.8
作者:
Fukase,M;Avioli,LV;Birge,SJ;Chase,LR
通讯作者: Chase,LR
甲状旁腺激素相关蛋白作为激素原:翻译后加工和受体相互作用。
DOI: 10.1210/edrv-15-1-40
发表时间: 1994
期刊: Endocrine reviews
影响因子: 20.3
作者:
Orloff,JJ;Reddy,D;dePapp,AE;Yang,KH;Soifer,NE;Stewart,AF
通讯作者: Stewart,AF
X 连锁低磷血症小鼠肾脏 25-羟基维生素 D-1 α-羟化酶活性的异常调节。
DOI: 10.1172/jci110783
发表时间: 1983
期刊: The Journal of clinical investigation
影响因子: --
作者:
Lobaugh,B;Drezner,MK
通讯作者: Drezner,MK