英文名稱：Human Intact FGF-23 ELISA Kit
【預(yù)期用途】
本產(chǎn)品僅供科研使用，主要用于測定血漿或細胞培養(yǎng)基中人全段成纖維細胞生長因子23。
【簡介】
成纖維細胞生長因子23（FGF-23）是最近發(fā)現(xiàn)的相關(guān)蛋白大家族中的新成員。其基因編碼一個含250個氨基酸殘基的蛋白。FGF-23的氨基-末端（aa 1-24）是疏水的且很可能作為其進入血液循環(huán)的信號肽。它的羧基-末端（aa 180-251）與FGF蛋白家族的其它成員僅有有限的同源性。FGF-23與FGF-21（~24% 為同源序列）和FGF-19（~22%為同源序列）最接近。
腎磷酸鹽流失可導(dǎo)致低血磷癥，是骨骼礦化和生長板發(fā)展缺陷的原因之一。常染色體顯性低血磷性佝僂�。�ADHR，一種罕見遺傳疾病）患者體內(nèi)攜帶一種FGF-23突變子，該突變子可使蛋白質(zhì)免受水解斷裂。而且，并發(fā)瘤原性骨軟化（OOM）的腫瘤患者被證實有過量的FGF-23 mRNA表達，似乎表明血液中FGF-23濃度的上升是導(dǎo)致這類病人磷酸鹽流失的原因。對嚙齒動物應(yīng)用重組FGF-23后發(fā)現(xiàn)尿中磷的排泄量增加進而導(dǎo)致低血鱗癥和骨軟化/佝僂病也印證了這一結(jié)論�？傊�，現(xiàn)有文獻表明FGF-23直接或間接地與磷酸鹽動態(tài)平衡的調(diào)節(jié)有關(guān)。
測定人體血液循環(huán)中的全段FGF-23水平可能為以下不同程度的血磷酸鹽流失障礙患者的實驗室評估提供重要的診斷工具：瘤原性骨軟化，X-鏈鎖低血磷性佝僂病和常染色體遺傳性低血磷性佝僂病。此外，對FGF-23的靈敏測定可能為骨骼調(diào)節(jié)和礦化動態(tài)提供新的見解。
【樣本要求】
鑒于全段FGF-23分子極不穩(wěn)定而導(dǎo)致免疫反應(yīng)活性隨時間降低，樣本收集、檢測和保存過程應(yīng)力求迅速。測定全段FGF-23濃度可使用EDTA血漿或細胞培養(yǎng)基作為樣本。要求雙份檢測，所需樣本量為300μL。
建議早晨空腹12小時后采血，對其離心分離出血漿，或分離細胞培養(yǎng)液獲得培養(yǎng)基。收集到的樣本應(yīng)馬上分析或冰凍保存在-20oC或更低溫度。避免反復(fù)凍融樣本。
【參考文獻】
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.
2. Jonsson KB, Zahradnik
R, Larsson T, White K, Sugimoto T, Imanishi Y, Yamamoto T, Hampson G, Koshiyama
H, Ljunggren Ö, Oba K, Yang IM, Miyauchi A, Econs MJ, Lavigne J, Jüppner H.
“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.
8. Weber TJ, Liu SG, Guo
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.