半岛bd体育手机客户端 说明
一般描述
OSTM1 (osteopetrosis associated transmembrane protein 1) is one of the genes associated with Autosomal Recessive Osteopetrosis (ARO) in humans. It was first identified in murine model, as the gene associated with grey-lethal mutant mice. This protein is thought to be an E3 ubiquitin ligase as well as a Gα-interacting protein. It is a type II transmembrane protein, which resides in intracellular compartments. In mice, this gene encodes a protein with predicted 338 amino acids. The region between N- and C- termini of the protein is hydrophobic, and shows slight homology to RING-finger proteins. It also has many putative N-linked glycosylation sites. OSTM1 is expressed in multiple tissues such as, osteoclasts, brain, liver and kidney.
免疫原
Osteopetrosis-associated transmembrane protein 1 precursor recombinant protein epitope signature tag (PrEST)
应用
Anti-OSTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
生化/生理作用
The exact function of OSTM1 (osteopetrosis associated transmembrane protein 1) gene is not yet known. However, it acts as a β subunit for the protein ClC-7 (Chloride channel 7), as it co-localizes with ClC-7 in lysosomes and late endosomes in multiple tissues, as well as in the bone-resorbing osteoclasts, in their ruffled borders. This gene is essential for the maturation and normal function of osteocytes and melanocytes. In humans, mutations in this gene lead to autosomal recessive osteopetrosis (ARO). ARO is a rare hereditary disorder, which is a result of oscteoclast resorption failure, and is characterized by bone marrow failure, increased bone density and fractures. OSTM1 is also responsible for normal neural development, and mutations in this gene lead to ARO with severe neuronopathy. This phenotype is characterized by malformations in the central nervous system, blindness, seizures, and deafness.
特点和优势
Prestige Antibodies?are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies?to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
联系
Corresponding Antigen APREST71674.
外形
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律信息
Prestige Antibodies is a registered trademark of sigma-aldrich Co. LLC
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
| 人类蛋白质图谱编号 | HPA010851 Human Protein Atlas characterization data |
半岛bd体育手机客户端 性质
| 生物来源 | rabbit |
| 质量水平 | 100 |
| 偶联物 | unconjugated |
| 抗体形式 | affinity isolated antibody |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 半岛bd体育手机客户端 线 | Prestige Antibodies?Powered by Atlas Antibodies |
| 形式 | buffered aqueous glycerol solution |
| species reactivity | human |
| 包装 | antibody small pack of 25 μL |
| 增强验证 | recombinant expression Learn more aboutAntibody Enhanced Validation |
| technique(s) | immunoblotting: 0.04-0.4 μg/mL immunohistochemistry: 1:200-1:500 |
| 免疫原序列 | REAYKTLSSLYSEMQKMNELENKAEPGTHLCIDVEDAMNITRKLWSRTFNCSVP |
| UniProt登记号 | Q86WC4 |
| 运输 | wet ice |
| 储存温度 | ?20℃ |
| Gene Information | human ... OSTM1(28962) |
安全信息
| 储存分类代码 | 12 - Non Combustible Liquids |
| WGK | WGK 1 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
| 个人防护装备 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
Sigma-Aldrich
m.cnreagent.com
