英文名称:Human Intact FGF-23 ELISA Kit
【预期用途】
本产品仅供科研使用,主要用于测定血浆或细胞培养基中人全段成纤维细胞生长因子23。
【简介】
成纤维细胞生长因子23(FGF-23)是最近发现的相关蛋白大家族中的新成员。其基因编码一个含250个氨基酸残基的蛋白。FGF-23的氨基-末端(aa 1-24)是疏水的且很可能作为其进入血液循环的信号肽。它的羧基-末端(aa 180-251)与FGF蛋白家族的其它成员仅有有限的同源性。FGF-23与FGF-21(~24% 为同源序列)和FGF-19(~22%为同源序列)最接近。
肾磷酸盐流失可导致低血磷症,是骨骼矿化和生长板发展缺陷的原因之一。常染色体显性低血磷性佝偻病(ADHR,一种罕见遗传疾病)患者体内携带一种FGF-23突变子,该突变子可使蛋白质免受水解断裂。而且,并发瘤原性骨软化(OOM)的肿瘤患者被证实有过量的FGF-23 mRNA表达,似乎表明血液中FGF-23浓度的上升是导致这类病人磷酸盐流失的原因。对啮齿动物应用重组FGF-23后发现尿中磷的排泄量增加进而导致低血鳞症和骨软化/佝偻病也印证了这一结论。总之,现有文献表明FGF-23直接或间接地与磷酸盐动态平衡的调节有关。
测定人体血液循环中的全段FGF-23水平可能为以下不同程度的血磷酸盐流失障碍患者的实验室评估提供重要的诊断工具:瘤原性骨软化,X-链锁低血磷性佝偻病和常染色体遗传性低血磷性佝偻病。此外,对FGF-23的灵敏测定可能为骨骼调节和矿化动态提供新的见解。
【样本要求】
鉴于全段FGF-23分子极不稳定而导致免疫反应活性随时间降低,样本收集、检测和保存过程应力求迅速。测定全段FGF-23浓度可使用EDTA血浆或细胞培养基作为样本。要求双份检测,所需样本量为300μL。
建议早晨空腹12小时后采血,对其离心分离出血浆,或分离细胞培养液获得培养基。收集到的样本应马上分析或冰冻保存在-20oC或更低温度。避免反复冻融样本。
【参考文献】
1. Shimada T, Muto T,
Urakawa I, Yoneya T, Yamazaki Y, Okawa K, Takeuchi Y, Fujita T, Fukumoto S,
Yamashita T. “Mutant FGF-23 responsible for autosomal dominant hypophosphatemic
rickets is resistant to proteolytic
cleavage and causes hypophosphatemia in vivo”. Endocrinology 143(8):3179-3182.
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R, Larsson T, White K, Sugimoto T, Imanishi Y, Yamamoto T, Hampson G, Koshiyama
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“Fibroblast Growth Factor 23 in Oncogenic Osteomalacia and X-Linked
Hypophosphatemia.” N Engl J Med 2003; 348:1656-63.
3. White KE, Evans WE,
O′Riordan JLH, Speer MC, Econs MJ, Group2.Lorenz-
Depiereux B, Grabowski M, Mettinger T,Strom TM. “Autosomal dominant hypophosphataemic
rickets is associated with mutations in FGF23”. Nat. Genet, 2000, 26:345-8.
4. Yamashita T, Yoshioka M, Itoh N. “Identification of a Novel Fibroblast Growth Factor, FGF-23, Preferentially Expressed in the Ventrolateral Thalamic Nucleus of the Brain.” Biochemical and Biophysical Research Communications, 2000, 277:494-98.
5. White KE, Jonsson KB, Carn G, Hampson G, Spector TD, Mannstadt M, Lorenz-Depiereux B, Miyauchi A, Yang IM, Ljunggren O, Mettinger T, Strom TM, Jueppner H, Econs MJ.“The AutosomalDominant Hypophosphatemic Rickets (ADHR)Gene Is a Secreted Polypeptide Overexpressed by Tumorsthat Cause Phosphate Wasting”. J. Clin. Endocrinol. Metab.,2001, 86:497-500.
6. Shimada T, Mizutani
S, Muto T, Yoneya T, Hino R, Takeda S,Takeuchi Y, Fujita T, Fukumoto S,
Yamashita T. “Cloning and Characterization of FGF23 As a Causative Factor of
Tumorinduced Osteomalacia”. Proc. Natl. Acad. Sci. USA, 2001,98:6500-05.
7. Strewler G. “FGF23,Hypophosphatemia,
and Rickets: Has Phosphatonin Been Found?”. Proc. Natl. Acad. Sci. USA, 2001,
98:5945-46.
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R, Simpson LG, Quarles LD.“Elevated FGF23 Serum Concentrations in XLH: An
Indirect Consequence of Inactivating Phex Mutations?” J Bone Miner
Res 2003; 18:387.
9. Preissner CM, Singh R, Kumar R. “Elevated Fibroblast Growth Factor 23 Concentrations in Humoral Hypercalcemia of Malignancy and Primary Hyperparathyroidism”. J Bone Miner Res 2002; 17:S150.