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货号： bs-8062R-PE-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8062R-PE-Cy3
- 英文名称
- Anti-PLEKHM1/PE-Cy3
- 中文名称
- PE-Cy3标记的石骨症相关蛋白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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Horse, Rabbit,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 117kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 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
- 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:
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.
Similarity:
Contains 2 PH domains.
Contains 1 phorbol-ester/DAG-type zinc finger.
Contains 1 RUN domain.
Database links:
UniProtKB/Swiss-Prot: Q9Y4G2.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.