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货号： bs-5081R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5081R-Cy7
- 英文名称
- Anti-Lamin B Receptor/Cy7
- 中文名称
- Cy7标记的核纤层蛋白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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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, Rat, Cow, Horse, Rabbit,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 68kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Lamin B Receptor
- 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
- 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.
- 产品介绍
- background:
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.
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.