| 產(chǎn)品編號(hào) | bs-12313R |
| 英文名稱 | PHEX Rabbit pAb |
| 中文名稱 | 維生素D低磷性佝僂病蛋白 |
| 別 名 | HPDR; HPDR1; HYP; HYP1; LXHR; Metalloendopeptidase homolog PEX; PEX; Phex; PHEX_HUMAN; Phosphate regulating endopeptidase homolog X linked; Phosphate regulating gene with homologies to endopeptidases on the X chromosome; Phosphate-regulating neutral endopeptidase; Vitamin D-resistant hypophosphatemic rickets protein; X-linked hypophosphatemia protein; XLH. |
| 研究領(lǐng)域 | 發(fā)育生物學(xué) 干細(xì)胞 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | Flow-Cyt=1ug/Test
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 86 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Human PHEX: 201-300/749 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The PHEX a 749 amino acid protein that putatively consists of an intracellular, transmembrane and extracellular domain. PHEX mutations have been observed in 60-80% of hypophosphatemic rickets patients. The PHEX protein, which is a single-pass membrane protein, is also designated HYP, X-linked hypophosphatemia protein or metalloendopeptidase homolog PEX. PHEX plays an active role in bone and dentin mineralization and renal phosphate re-absorption. X-linked hypophosphatemic rickets, also designated HYP, is an X-linked dominant disorder characterized by impaired phosphate uptake in the kidney, which is likely to be caused by abnormal regulation of sodium phosphate cotransport in the proximal tubules. Clinical manifestations include skeletal deformities, growth failure, craniosynostosis, paravertebral calcifications, pseudofractures in lower extremities, and muscular hypotonia with onset in early childhood. Function: Probably involved in bone and dentin mineralization and renal phosphate reabsorption. Subcellular Location: Membrane. Tissue Specificity: Lymphocytes and fetal brain; not in adult brain, placenta, skeletal muscle and pancreas; not in adult and fetal heart, lung, liver and kidney. DISEASE: Defects in PHEX are a cause of X-linked hypophosphatemic rickets (HYP) [MIM:307800]. HYP is an X-linked dominant disorder characterized by impaired phosphate uptake in the kidney, which is likely to be caused by abnormal regulation of sodium phosphate cotransport in the proximal tubules. Clinical manifestations include skeletal deformities, growth failure, craniosynostosis, paravertebral calcifications, pseudofractures in lower extremities, and muscular hypotonia with onset in early childhood. X-linked hypophosphatemic rickets is the most common form of hypophosphatemia with an incidence of 1 in 20000. Similarity: Belongs to the peptidase M13 family. SWISS: P78562 Gene ID: 5251 Database links: Entrez Gene: 5251 Human Omim: 300550 Human SwissProt: P78562 Human Unigene: 495834 Human |
| 產(chǎn)品圖片 |
Blank control: A549.
Primary Antibody (green line): Rabbit Anti-PHEX antibody (bs-12313R)
Dilution: 1μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody : Goat anti-rabbit IgG-PE
Dilution: 1μg /test.
Protocol
The cells were incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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