石骨症相关蛋白PLEKHM1抗体

石骨症相关蛋白PLEKHM1抗体

规格：1mg/1ml

英文名： PLEKHM1

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.

分子量： 117kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human PLEKHM1

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

细胞定位：细胞浆

石骨症相关蛋白PLEKHM1抗体产品介绍：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 *****hood. 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: 石骨症相关蛋白PLEKHM1抗体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, 石骨症相关蛋白PLEKHM1抗体or childhood, and a benign autosomal dominant formoccurring in adolescence or *****hood. 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.

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.

研究领域：细胞生物  **学  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid

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