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產品規格
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BN41039R-100ul
100ul
￥2360.00
交叉反應：Mouse,Human(predicted:Rabbit,Horse,Cow,Rat) 推薦應用：WB,ICC,Flow-Cyt,ELISA
BN41039R-200ul
200ul
￥3490.00
交叉反應：Mouse,Human(predicted:Rabbit,Horse,Cow,Rat) 推薦應用：WB,ICC,Flow-Cyt,ELISA
產品描述
| 英文名稱 | Lamin B Receptor |
| 中文名稱 | 核纖層蛋白B受體抗體 |
| 別 名 | LBR_HUMAN; LMN2R; PHA; DHCR 14B; DHCR14B antibody Integral nuclear envelope inner membrane protein; Lamin-B receptor; LBR; LMN 2R; LMN2R; MGC9041; PHA; PRO0650; DHCR14B; MGC9041. |
| 研究領域 | 腫瘤 細胞生物 免疫學 神經生物學 信號轉導 轉錄調節因子 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Human, Mouse, (predicted: Rat, Cow, Horse, Rabbit, ) |
| 產品應用 | WB=1:500-2000 ELISA=1:5000-10000 Flow-Cyt=1ug/Test not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 68kDa |
| 細胞定位 | 細胞核 細胞漿 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Lamin B Receptor:1-100/615 |
| 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 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產品介紹 | Lamins are nuclear membrane proteins that serve to maintain specific cellular functions, such as DNA replication and chromatin organization. Lamin B receptor (LBR) is an integral protein of the nuclear envelope inner membrane. It is phosphorylated by CDC2 protein kinase in mitosis when the inner nuclear membrane breaks down into vesicles that dissociate from the lamina and the chromatin. It is phosphorylated by different protein kinases in interphase when the membrane is associated with these structures. The cleavage of lamins results in nuclear disregulation and cell death. Function: Anchors the lamina and the heterochromatin to the inner nuclear membrane. Subunit: Interacts directly with CBX5. Can interact with chromodomain proteins. Interacts directly with DNA. Interaction with DNA is sequence independent with higher affinity for supercoiled and relaxed circular DNA than linear DNA. Subcellular Location: Nucleus inner membrane; Multi-pass membrane protein. Post-translational modifications: Phosphorylated by CDK1 in mitosis when the inner nuclear membrane breaks down into vesicles that dissociate from the lamina and the chromatin. It is phosphorylated by different protein kinases in interphase when the membrane is associated with these structures. Phosphorylation of LBR and HP1 proteins may be responsible for some of the alterations in chromatin organization and nuclear structure which occur at various times during the cell cycle. Phosphorylated by SRPK1. In late anaphase LBR is dephosphorylated, probably by PP1 and/or PP2A, allowing reassociation with chromatin. DISEASE: Defects in LBR are a cause of Pelger-Huet anomaly (PHA) [MIM:169400]. PHA is an autosomal dominant inherited abnormality of neutrophils, characterized by reduced nuclear segmentation and an apparently looser chromatin structure. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin. Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy, and skeletal abnormalities. [DISEASE] Defects in LBR are the cause of hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM) [MIM:215140]; also known as Greenberg skeletal dysplasia. HEM is a rare autosomal recessive chondrodystrophy characterized by early in utero lethality and, therefore, considered to be nonviable. Affected fetuses typically present with fetal hydrops, short-limbed dwarfism, and a marked disorganization of chondro-osseous calcification and may present with polydactyly and additional nonskeletal malformations. Similarity: Belongs to the ERG4/ERG24 family. Contains 1 Tudor domain. SWISS: Q14739 Gene ID: 3930 Database links: Entrez Gene: 3930 Human Omim: 600024 Human SwissProt: Q14739 Human Unigene: 435166 Human Unigene: 6499 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. [DISEASE] Defects in LBR may be a cause of Reynolds syndrome (REYNS) [MIM:613471]. It is a syndrome specifically associating limited cutaneous systemic sclerosis and primary biliray cirrhosis. It is characterized by liver disease, telangiectasia, abrupt onset of digital paleness or cyanosis in response to cold exposure or stress (Raynaud phenomenon), and variable features of scleroderma. The liver disease is characterized by pruritis, jaundice, hepatomegaly, increased serum alkaline phosphatase and positive serum mitochondrial autoantibodies, all consistent with primary biliary cirrhosis. |
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