產(chǎn)品編號(hào) | bs-18310R-BF594 |
英文名稱 | Rabbit Anti-LMBRD2/BF594 Conjugated antibody |
中文名稱 | BF594標(biāo)記的LMBRD2蛋白抗體 |
別 名 | LMBD2_HUMAN; LMBR1 domain-containing protein 2; LMBRD2. |
規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
說(shuō) 明 書 | 100ul |
研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) |
抗體來(lái)源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Horse, Rabbit, ) |
產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 81kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human LMBRD2 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
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. |
產(chǎn)品介紹 |
background: Vitamin B12 (cobalamin) is essential in animals and humans for metabolism of methylmalonic acid, for the remethylation of homocysteine to methionine and, consequently, for all S-adenosylmethionine-dependent methylation reactions, including DNA synthesis. The lysosomal cobalamin transporter is required for the export cobalamin from lysosomes allowing its conversion to cofactors. Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAFHC), also known as homocystinuria-megaloblastic anemia complementation type F. MMAFHC is a disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl) due to accumulation of cobalamin in lysosomes. Clinical features of MMAFHC include developmental delay, stomatitis, glossitis, seizures and methylmalonic aciduria in response to vitamin B12. LMBRD2 (LMBR1 domain containing 2) is a 695 amino acid multi-membrane protein that may have similar functions as LMBR1. Subcellular Location: Membrane. Similarity: Belongs to the LIMR family. Database links: Entrez Gene: 92255 Human SwissProt: Q68DH5 Human Unigene: 294103 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |