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Lamin B Receptor Rabbit pAb (bs-5081R)  
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產(chǎn)品編號(hào) bs-5081R
英文名稱 Lamin B Receptor Rabbit pAb
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(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse (predicted: Rat,Rabbit,Cow,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000,Flow-Cyt=1ug/Test,ICC/IF=1:25
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 68 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞核 細(xì)胞漿 細(xì)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
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)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

Entrez Gene: 89789 Rat

Omim: 600024 Human

SwissProt: Q14739 Human

SwissProt: O08984 Rat

Unigene: 435166 Human

Unigene: 6499 Rat



[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.
產(chǎn)品圖片
Sample: Jurkat(Human) Cell Lysate at 30 ug Primary: Anti-Lamin B Receptor (bs-5081R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 68 kD Observed band size: 68 kD
Sample: Hela(Human) Cell Lysate at 30 ug Primary: Anti-Lamin B Receptor (bs-5081R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 68 kD Observed band size: 68 kD
Hela cell; 4% Paraformaldehyde-fixed; Triton X-100 at room temperature for 20 min; Blocking buffer (normal goat serum, C-0005) at 37°C for 20 min; Antibody incubation with (Lamin B Receptor) polyclonal Antibody, Unconjugated (bs-5081R) 1:25, 90 minutes at 37°C; followed by a conjugated Goat Anti-Rabbit IgG antibody at 37°C for 90 minutes, DAPI (blue, C02-04002) was used to stain the cell nuclei.
Blank control (Black line):Molt4 (Black). Primary Antibody (green line): Rabbit Anti-Lamin B Receptor antibody (bs-5801R) Dilution: 1μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody (white blue line): Goat anti-rabbit IgG-AF647 Dilution: 1μg /test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at room temperature. The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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