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重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H ELISA, WB, ICC M Purified Monoclonal Antibody 描述 产品目录编号 05-1231 商名 - Upstate
描述 Anti-Progerin Antibody, clone 13A4 Alternate Names - CMD1A
- LMNC
- LMN1
- LDP1
- LFP
- FPLD
- FPL
- EMD2
- LMNA
背景信息 Progerin is a truncated version of lamin A protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a mutation (C1824T) in the lamin A gene, LMNA. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a deletion of 50 amino acids. Lamin A constitutes a major structural component of the lamina, a scaffold of proteins found inside the nuclear membrane of a cell; progerin does not properly integrate into the lamina, which disrupts the scaffold structure and leads to significant disfigurement of the nucleus, characterized by a lobular shape. Researchers have shown that progerin activates genes that regulate stem cell differentiation via the Notch signaling pathway. Progerin, which has been linked to normal aging, is produced in healthy indivduals via "sporadic use of the cryptic splice site. 产品信息 格式 Purified 控制 - HeLa cells expressing human progerin
演示 Protein G purified monoclonal antibody from ascites. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide. 应用 应用 This Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin. 主要应用 - ELISA
- Western Blotting
- Immunocytochemistry
生物信息 免疫原品种 KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C). 克隆 13A4 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C 同种型 IgG1κ 品种反应性 Human Species Reactivity Note Human. Other species not tested. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_005563
Entrez基因汇总 The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Through alternate splicing, this gene encodes three type A lamin isoforms. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome. [provided by RefSeq].
基因符号 - LMNA
纯化方法 Protein G purfied UniProt编号 - Q6UYC3
产品使用声明 质量保证 Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin. 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt.
For maximum recovery of product, centrifuge the vial prior to removing the cap.
包装信息 数量 100 µg
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