FITC标记的维生素D低磷性佝偻病蛋白

FITC标记的维生素D低磷性佝偻病蛋白

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  • ¥2980
  • LMAI Bio
  • LM-12313R-FITC
  • 中国/美国/欧洲
  • 2025年07月04日
  • ICC=1:50-200 IF=1:50-200
  • Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
  • Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
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    • 详细信息
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 靶点

      详见说明书

    • 级别

      1

    • 目录编号

      LM-12313R-FITC

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      1年

    • 抗体英文名

      Anti-PHEX/FITC

    • 抗体名

      Anti-PHEX/FITC

    • 标记物

      FITC标记

    • 宿主

      Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

    • 适应物种

      Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

    • 免疫原

      详见说明书

    • 亚型

      IGg

    • 形态

      粉末、液体、冻干粉

    • 应用范围

      ICC=1:50-200 IF=1:50-200

    • 浓度

      1mg/ml

    • 保存条件

      -20 °C

    • 规格

      100ul

    FITC标记的维生素D低磷性佝偻病蛋白
    英文名称 Anti-PHEX/FITC
    中文名称 FITC标记的维生素D低磷性佝偻病蛋白
    别    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.  
    规格价格 100ul/2980元 购买        大包装/询价
    说 明 书 100ul  
    研究领域 发育生物学  干细胞  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
    产品应用 ICC=1:50-200 IF=1:50-200  
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 86kDa
    细胞定位 细胞膜 
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from Human PHEX
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    产品介绍 background:
    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.

    Database links:
    UniProtKB/Swiss-Prot: P78562.1

    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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