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货号： bs-1841R-Cy5.5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-1841R-Cy5.5
- 英文名称
- Anti-lamin A + C/Cy5.5
- 中文名称
- Cy5.5标记的核纤层蛋白A/C抗体
- 别 名
- lamin A/C; lamin A+C; FPL; FPLD; HGPS; IDC; Lamin A/C; LaminC; LDP1; LGMD1B; LMN 1; LMN C; PRO1; LMNA_HUMAN; Prelamin-A/C; Lamin-A/C; 70 kDa lamin; Renal carcinoma antigenNY-REN-32.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 细胞凋亡
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 73kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human lamin A/C
- 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:
An important part of the cell nucleus is formed by nuclear lamina. Nuclear lamins form a network of filaments at the nucleoplasmic site of the nuclear membrane. Two main subtypes of nuclear lamins can be distinguished, i.e. A-type lamins and B-type lamins. The A-type lamins comprise a set of three proteins arising from the same gene by alternative splicing, i.e. lamin A, lamin C and lamin Adel10, while the B-type lamins include two proteins arising from two distinct genes, i.e. lamin B1 and lamin B2.
The nuclear lamins comprise a unique subclass of the intermediate filament protein family. They share a molecular domain organisation with the other intermediate filament proteins in that they are fibrous molecules that have an aminoterminal globular head, a central rod of a-helices and a carboxyterminal globular domain.
Many biochemical and molecular features of lamins have been studied, but their functions remain still largely undetermined. One of the functions ascribed to the lamina is the maintenance of the structural integrity of the nucleus.
Besides interactions with the nuclear membrane and other intermediate filaments, lamins interact with the nuclear chromatin. Eukaryotic chromatin is organised into loops, which are attached to the nuclear matrix. This organisation is thought to contribute to compaction of the chromatin and regulation of gene expression. Lamins, as part of the nuclear matrix, may be involved in these processes since chromatin binding sites have been detected in both A- and B-type lamins.
Function:
Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. Play an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics.
Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence
Subunit:
Homodimer of lamin A and lamin C. Interacts with lamin-associated polypeptides IA, IB and TMPO-alpha, RB1 and with emerin. Interacts with SREBF1, SREBF2, SUN2 and TMEM43. Proteolytically processed isoform A interacts with NARF. Interacts with SUN1. Prelamin-A/C interacts with EMD. Interacts with MLIP; may regulate MLIP localization to the nucleus envelope. Interacts with DMPK; may regulate nuclear envelope stability.
Subcellular Location:
Nucleus. Nucleus envelope.
Tissue Specificity:
In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress.
Post-translational modifications:
Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations.
Proteolytic cleavage of the C-terminal of 18 residues of prelamin-A/C results in the production of lamin-A/C. The prelamin-A/C maturation pathway includes farnesylation of CAAX motif, ZMPSTE24/FACE1 mediated cleavage of the last three amino acids, methylation of the C-terminal cysteine and endoproteolytic removal of the last 15 C-terminal amino acids. Proteolytic cleavage requires prior farnesylation and methylation, and absence of these blocks cleavage.
Sumoylation is necessary for the localization to the nuclear envelope.
Farnesylation of prelamin-A/C facilitates nuclear envelope targeting.
DISEASE:
Defects in LMNA are the cause of Emery-Dreifuss muscular dystrophy type 2, autosomal dominant (EDMD2) [MIM:181350]. A degenerative myopathy characterized by weakness and atrophy of muscle without involvement of the nervous system, early contractures of the elbows, Achilles tendons and spine, and cardiomyopathy associated with cardiac conduction defects.
Database links:Entrez Gene: 4000 Human
Entrez Gene: 16905 Mouse
Entrez Gene: 60374 Rat
Omim: 150330 Human
SwissProt: P02545 Human
SwissProt: P48678 Mouse
SwissProt: P48679 Rat
Unigene: 594444 Human
Unigene: 243014 Mouse
Unigene: 471227 Mouse
Unigene: 44161 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
核膜标志物（Nuclear Envelope Marker）
核纤层蛋白(lamin) 是紧贴核内膜的一层厚度为20～50nm的纤维蛋白层或纤维网络。核纤层与细胞质骨架、核骨架连成一个整体，一般认为核纤层将核被膜和染色质提供了结构支架。有学者研究认为：lamin蛋白与细胞凋亡及衰老有关联，它包括：核纤层蛋白A、核纤层蛋白B、核纤层蛋白C几个不同亚型的蛋白。