Special Offers
100% Performance Guaranteed
Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB, ICC, ELISA, IP | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | 05-1231 |
| Trade Name |
|
| Description | Anti-Progerin Antibody, clone 13A4 |
| Alternate Names |
|
| Background Information | 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. |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Protein G purified immunoglobulin. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin. |
| Key Applications |
|
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C). |
| Clone | 13A4 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C |
| Isotype | IgG1κ |
| Species Reactivity |
|
| Species Reactivity Note | Human. Other species not tested. |
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | 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]. |
| Gene Symbol |
|
| Purification Method | Protein G Purified |
| UniProt Number |
|
| Molecular Weight | 70 kDa |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | 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. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |