PLEKHM1,石骨症相关蛋白PLEKHM1抗体

产品编号HZ-8062R
英文名称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.
说 明 书0.1ml 0.2ml
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,
PLEKHM1,石骨症相关蛋白PLEKHM1抗体产品应用WB=1:100-500 ELISA=1:500-1000 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.
分 子 量117kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
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.
PLEKHM1,石骨症相关蛋白PLEKHM1抗体PubMedPubMed
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.

PLEKHM1,石骨症相关蛋白PLEKHM1抗体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

PLEKHM1,石骨症相关蛋白PLEKHM1抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Paraformaldehyde-fixed, paraffin embedded (mouse placenta); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PLEKHM1) Polyclonal Antibody, Unconjugated (HZ-8062R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.