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病相關(guān)跨膜蛋白1抗體規(guī)格 英文名稱  Anti-OSTM1

中文名稱  病相關(guān)跨膜蛋白1抗體規(guī)格 

別    名 GAIP-interacting protein N terminus; GIPN; GL antibody Grey lethal osteopetrosis; HSPC019; OPTB5; Osteopetrosis-associated transmembrane protein 1; Ostm1; OSTM1_HUMAN.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來(lái)源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Pig

產(chǎn)品類型   一抗  

研究領(lǐng)域    

蛋白分子量  predicted molecular weight: 33kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human OSTM1

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

病相關(guān)跨膜蛋白1抗體規(guī)格 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-100 IF=1:50-200

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 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.

Subcellular Location : Membrane; Single-pass type I membrane protein.

[DISEASE] Defects in OSTM1 are the cause of osteopetrosis autosomal recessive type 5 (OPTB5) ; 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.

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技術(shù)外包服務(wù):

★病相關(guān)跨膜蛋白1抗體規(guī)格 分子生物學(xué)：質(zhì)粒抽提、PCR、Q-PCR、RT-PCR、分子生物學(xué)：基因合成、引物合成、基因測(cè)序、載體構(gòu)建等

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★病毒包裝：腺病毒、慢病毒等

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★細(xì)胞工程：細(xì)胞表型分析(凋亡、增殖、周期、遷移、侵襲、修復(fù)、克隆形成)、細(xì)胞培養(yǎng)、細(xì)胞膜制備、穩(wěn)定細(xì)胞株構(gòu)建、細(xì)胞RNAi技術(shù)等等。