产品编号 | bs-8506R-Gold |
英文名称 | Anti-OSTM1/Gold |
中文名称 | 胶体金标记的骨硬化病相关跨膜蛋白1抗体 |
别 名 | GAIP-interacting protein N terminus; GIPN; GL antibody Grey lethal osteopetrosis; HSPC019; OPTB5; Osteopetrosis-associated transmembrane protein 1; Ostm1; OSTM1_HUMAN. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul(10nm 15nm 35nm) |
研究领域 | |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | (predicted: Human, Mouse, Rat, Pig, ) |
产品应用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 33kDa |
性 状 | Lyophilized or Liquid |
浓 度 | 0.4mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human OSTM1 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
储 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
保存条件 | Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles. |
产品介绍 |
background: OSTM1 (osteopetrosis associated transmembrane protein 1), also known as gl (gray-lethal) or HSPC019, is a 338 amino acid single-pass type I membrane protein that is expressed primarily in osteoclasts and melanocytes as well as brain, kidney and spleen. Bone autosomal recessive osteopetrosis (ARO) is the most severe form of hereditary bone disease whose cellular basis is in the osteoclast and is characterized by abnormally dense bone, due to defective resorption of immature bone. ARO is suggested to be caused by mutations in the OSTM1 gene. 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. Defects in the OSTM1 gene are also the cause of the spontaneous gl mutant, which is responsible for a coat color defect in mice. Function: Required for osteoclast and melanocyte maturation and function (By similarity). Subcellular Location: Membrane; Single-pass type I membrane protein. DISEASE: Defects in OSTM1 are the cause of osteopetrosis autosomal recessive type 5 (OPTB5) [MIM:259720]; also called infantile malignant osteopetrosis 3. 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. OPTB5 patients manifest primary central nervous system involvement in addition to the classical stigmata of severe bone sclerosis, growth failure, anemia, thrombocytopenia and visual impairment with optic atrophy. Database links: UniProtKB/Swiss-Prot: Q86WC4.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |