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首页>>免疫学>>一抗>>核纤层蛋白B受体抗体
核纤层蛋白B受体抗体
  • 产品货号：
    BN41039R
  • 中文名称：
    核纤层蛋白B受体抗体
  • 英文名称：
    Rabbit anti-Lamin B Receptor Polyclonal antibody
  • 品牌：
    Biorigin

  • 货号

    产品规格

    售价

    备注

  • BN41039R-100ul

    100ul

    ¥2360.00

    交叉反应：Mouse,Human(predicted:Rabbit,Horse,Cow,Rat) 推荐应用：WB,ICC,Flow-Cyt,ELISA

  • BN41039R-200ul

    200ul

    ¥3490.00

    交叉反应：Mouse,Human(predicted:Rabbit,Horse,Cow,Rat) 推荐应用：WB,ICC,Flow-Cyt,ELISA

产品描述

英文名称Lamin B Receptor
中文名称核纤层蛋白B受体抗体
别    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.  
研究领域肿瘤  细胞生物  免疫学  神经生物学  信号转导  转录调节因子  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse,  (predicted: Rat, Cow, Horse, Rabbit, )
产品应用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
细胞定位细胞核 细胞浆 细胞膜 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Lamin B Receptor:1-100/615 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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



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.