產(chǎn)品編號(hào) | bs-11780R-BF488 |
英文名稱 | Rabbit Anti-LMX1b/BF488 Conjugated antibody |
中文名稱 | BF488標(biāo)記的指甲髕骨綜合征相關(guān)蛋白NPS1抗體 |
別 名 | LIM homeo box transcription factor 1 beta; LIM homeobox transcription factor 1 beta; LIM homeobox transcription factor 1-beta; LIM-homeobox protein 1.2; LIM/homeobox protein 1.2; LIM/homeobox protein LMX1B; LMX 1.2; LMX-1.2; LMX1.2; LMX1B; LMX1B_HUMAN; NPS 1; NPS1. |
規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
說 明 書 | 100ul |
研究領(lǐng)域 | 細(xì)胞生物 發(fā)育生物學(xué) 神經(jīng)生物學(xué) 表觀遺傳學(xué) |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | Mouse, Rat, (predicted: Human, Dog, Pig, Cow, 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 | 42kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human LMX1b/NPS1 |
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: This gene encodes a member of LIM-homeodomain family of proteins containing two N-terminal zinc-binding LIM domains, 1 homeodomain, and a C-terminal glutamine-rich domain. It functions as a transcription factor, and is essential for the normal development of dorsal limb structures, the glomerular basement membrane, the anterior segment of the eye, and dopaminergic and serotonergic neurons. Mutations in this gene are associated with nail-patella syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010] Function: Essential for the specification of dorsal limb fate at both the zeugopodal and autopodal levels. Subcellular Location: Nucleus. Tissue Specificity: Expressed in most tissues. Highest levels in testis, thyroid, duodenum, skeletal muscle, and pancreatic islets. DISEASE: Defects in LMX1B are the cause of nail-patella syndrome (NPS) [MIM:161200]; also known as onychoosteodysplasia. NPS is a disease that cause abnormal skeletal patterning and renal dysplasia. Similarity: Contains 1 homeobox DNA-binding domain. Contains 2 LIM zinc-binding domains. Database links: Entrez Gene: 4010 Human Entrez Gene: 16917 Mouse GenBank: U77457.1 Human Omim: 602575 Human SwissProt: O60663 Human SwissProt: O88609 Mouse Unigene: 129133 Human Unigene: 39825 Mouse Unigene: 92364 Rat 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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