| 货号 | 39966 |
| 克隆类别 | 13A4 |
| 同种亚型 | IgG1k |
| 反应种属 | Human |
| 来源宿主 | Mouse |
| 应用 | Western Blot/Immunofluorescence/Immunocytochemistry |
| 使用方法 | Published Applications: ICC/IF WB See references for more information. Individual optimization may be required. |
| 供应商 | Active Motif |
| 纯化方式 | Protein G Chromatography |
| 免疫原 | This Progerin antibody was raised against a peptide corresponding to amino acids 604-611 of human Progerin. |
| 背景 | Progerin is a 614 amino acid protein related to the Lamin A protein and is involved in Hutchinson-Gilford progeria syndrome (HGPS), a rare genetic disorder characterized by features reminiscent of severe premature aging. Progerin is most often generated by a point mutation in the gene that codes for lamin A and C. Nuclear lamins are intermediate filament proteins that are the major structural component of the nuclear lamina on the inner surface of the nuclear envelope. The mutation of the lamin A gene that leads to preogerin results in the creation of a cryptic splice site in lamin A mRNA, resulting in the deletion of 50 amino acids in the C-terminal region the lamin A protein. The 50 amino acid deletion removes a conserved proteolytic cleavage site and therefore Progerin retains its C-terminal 15 amino acids and is permanently farnesylated. Although it can enter the nucleus and associate with the nuclear envelope, it cannot incorporate normally into the nuclear lamina. HGPS is caused by the toxic accumulation of Progerin, which acts to deregulate mitosis and DNA damage signaling, leading to premature senescence and cell death. |
| 运输条件 | Blue Ice |
| 存放说明 | Antibodies in solution can be stored at -20°C for 2 years. Avoid repeated freeze/thaw cycles and keep on ice when not in storage. |
| 存储溶液 | Purified IgG in 70 mM Tris (pH 8), 105 mM NaCl, 31 mM glycine, 0.07 mM EDTA, 30% glycerol and 0.035% sodium azide. Sodium azide is highly toxic. |
| 计算分子量 | 69 kDa |
| 参考文献 |
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