石骨症相关蛋白PLEKHM1抗体

公司石骨症相关蛋白PLEKHM1抗体具有高纯度、高效价、高特异性的特点，仅用于科学研究，不可用于临床诊断及药物治疗，可以应用于多种实验。CST、Santa抗体品牌。

英文名称  Anti-PLEKHM1 

中文名称  石骨症相关蛋白PLEKHM1抗体 

别    名  162 kDa adapter protein; AP162; PH domain-containing family M member 1; PKHM1_HUMAN; Pleckstrin homology domain-containing family M member 1; PLEKHM1. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg
石骨症相关蛋白PLEKHM1抗体的相关产品:

Rabbit Anti-human IgG F(ab')2/APC  APC标记的兔抗人IgG F(ab')2 0.1ml

Rabbit Anti-human IgG F(ab')2/Bio  生物素标记的兔抗人IgG F(ab')2 0.1ml

Rabbit Anti-human IgG F(ab')2/Cy3  Cy3标记的兔抗人IgG F(ab')2 0.1ml 

石骨症相关蛋白PLEKHM1抗体抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Pig, Horse, Rabbit

产品类型  一抗    

研究领域  细胞生物 免疫学  

蛋白分子量  predicted molecular weight: 117kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human PLEKHM1 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

产品介绍 Involved in vesicular transport in the osteoclast (By similarity). May have a role in sialyl-lex-mediated transduction of apoptotic signals.

Tissue specificity: Expressed in placenta, liver, prostate, thymus, spleen, ovary, colon, colon carcinoma and peripheral blood lymphocytes (PBL). Weakly expressed in brain, lung, kidney, and testis. No expression in heart, skeletal muscle, pancreas and small intestine. Predominantly expressed in the breast carcinoma cell line MCF-7.

Involvement in disease:Defects in PLEKHM1 are the cause of osteopetrosis autosomal recessive type 6 (OPTB6); also known as autosomal recessive osteopetrosis intermediate form. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood. Autosomal recessive osteopetrosis is usually associated with normal or elevated amount of non-functional osteoclasts.

Function : Involved in vesicular transport in the osteoclast (Bysimilarity). May have a role in sialyl-lex-mediated transduction ofapoptotic signals.

Subunit : In colon carcinoma and breast carcinoma cells, itinteracts with sialyl-lex-positive protein.

Subcellular Location : Cytoplasm (Potential).

Tissue Specificity : Expressed in placenta, liver, prostate,thymus, spleen, ovary, colon, colon carcinoma and peripheral bloodlymphocytes (PBL). Weakly expressed in brain, lung, kidney, andtestis. No expression in heart, skeletal muscle, pancreas and smallintestine. Predominantly expressed in the breast carcinoma cellline MCF-7.

DISEASE : Defects in PLEKHM1 are the cause of osteopetrosisautosomal recessive type 6 (OPTB6) [MIM:611497]; also known asautosomal recessive osteopetrosis intermediate form. Osteopetrosisis a rare genetic disease characterized by abnormally dense bone,due to defective resorption of immature bone. The disorder occursin two forms: a severe autosomal recessive form occurring in utero,infancy, or childhood, and a benign autosomal dominant formoccurring in adolescence or adulthood. Autosomal recessiveosteopetrosis is usually associated with normal or elevated amountof non-functional osteoclasts.

石骨症相关蛋白PLEKHM1抗体Similarity : Contains 2 PH domains.

Contains 1 phorbol-ester/DAG-type zinc finger.

Contains 1 RUN domain. 

Database links : UniProtKB/Swiss-Prot: Q9Y4G2.3

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