產(chǎn)品編號 | bs-18343R-BF594 |
英文名稱 | Rabbit Anti-LOXHD1/BF594 Conjugated antibody |
中文名稱 | BF594標(biāo)記的脂氧合酶同源結(jié)構(gòu)域1抗體 |
別 名 | DFNB77; FLJ32670; LH2D1; Lipoxygenase homology domain-containing protein 1; Lipoxygenase homology domains 1; LOXH1_HUMAN; LOXHD1. |
規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
說 明 書 | 100ul |
研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 細(xì)胞膜蛋白 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, ) |
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 | 222kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human LOXHD1 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 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: This gene encodes a highly conserved protein consisting entirely of PLAT (polycystin/lipoxygenase/alpha-toxin) domains, thought to be involved in targeting proteins to the plasma membrane. Studies in mice show that this gene is expressed in the mechanosensory hair cells in the inner ear, and mutations in this gene lead to auditory defects, indicating that this gene is essential for normal hair cell function. Screening of human families segregating deafness identified a mutation in this gene which causes DFNB77, a progressive form of autosomal-recessive nonsyndromic hearing loss (ARNSHL). Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Mar 2010] Function: Involved in hearing. Required for normal function of hair cells in the inner ear. DISEASE: Defects in LOXHD1 are the cause of deafness autosomal recessive type 77 (DFNB77) [MIM:613079]. A form of non-syndromic deafness characterized by preserved low-frequency hearing, and a trend toward mild to moderate mid-frequency and high-frequency hearing loss during childhood and adolescence. Hearing loss progresses to become moderate to severe at mid and high frequencies during adulthood. Similarity: Contains 14 PLAT domains. Database links: Entrez Gene: 125336 Human Omim: 613072 Human SwissProt: Q8IVV2 Human Unigene: 345877 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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