| 產(chǎn)品編號(hào) | bs-0822R-BF647 |
| 英文名稱1 | Rabbit Anti-TARDBP/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的Tar DNA 結(jié)合蛋白43/TDP-43抗體 |
| 別 名 | TAR DNA-binding protein 43; TAR DNA binding protein 43; ALS10; OTTHUMP00000002171; TAR DNA binding protein 43; TAR DNA binding protein; TDP 43; TDP-43; TDP43; TARDBP; tar DNA binding protein; ALS10; TADBP_HUMAN. |
| 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 | 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 細(xì)菌及病毒 表觀遺傳學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Cow, Rabbit, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 45kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human TDP-43 |
| 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: HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20. [provided by RefSeq, Jul 2008] Function: DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR. Subcellular Location: Nucleus. In patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis, it is absent from the nucleus of affected neurons but it is the primary component of cytoplasmic ubiquitin-positive inclusion bodies. Tissue Specificity: Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen. Post-translational modifications: Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. DISEASE: Defects in TARDBP are the cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:612069]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. Similarity: Contains 2 RRM (RNA recognition motif) domains. Database links: Entrez Gene: 23435 Human Entrez Gene: 230908 Mouse Omim: 605078 Human SwissProt: Q13148 Human SwissProt: Q921F2 Mouse Unigene: 300624 Human Unigene: 635053 Human Unigene: 22453 Mouse Unigene: 2633 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 變異蛋白質(zhì)TDP-43 在額顳葉退行性病變（FTLD-U）和萎縮性側(cè)索硬化癥（ALS）中表達(dá)較高。TDP-43在大腦中堆積能導(dǎo)致神經(jīng)細(xì)胞衰竭,從而引發(fā)疾病肌萎縮性側(cè)索硬化（ALS，也被稱為L(zhǎng)ou Gehrig氏病） TDP-43這種癡呆是由大腦額葉的退化引起的，退化能延伸到顳葉。這是僅次于阿爾默海茲癥的讓65歲以下患者癡呆的第二種最常見的原因，通常影響40幾歲和50幾歲的人。TDP-43過(guò)去在神經(jīng)退化疾病患者病理的錯(cuò)誤折疊蛋白中缺失。識(shí)別出這個(gè)蛋白應(yīng)該有助于癡呆以及運(yùn)動(dòng)神經(jīng)元疾病的研究。 |
