Cat.No.: | EAb-1851 |
Product Name: | Progerin Monoclonal Antibody |
Antibody Type: | Monoclonal |
Immunogen: | This Progerin antibody was raised against a peptide corresponding to amino acids 604-611 of human Progerin. |
Host: | Mouse |
Isotype: | IgG1k |
Molecular Weight: | 69 kDa |
Purification: | Protein G Chromatography |
Appearance: | Liquid |
Formulation: | 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. |
Applications: | ICC/IF; WB |
Species Reactivity: | Human |
Storage: | -20°C |
Warning: | For Research Use Only! Not For Use in Humans. |
Scientific Background: | 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. |
Product Types | ||
◆ Bioactive Small Molecules | ||
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BSM-0047 | SGC707 | Inquiry |
◆ Antibodies | ||
EAb-0048 | PRMT6 Polyclonal Antibody | Inquiry |
EAb-0049 | PRMT7 Polyclonal Antibody | Inquiry |
EAb-0050 | PRMT1 Polyclonal Antibody | Inquiry |
Related Gene / Proteins | |||
Pr-SET7 | PRC1 | PRC2 | PRDM1 |
PRDM10 | PRDM11 | PRDM12 | PRDM13 |
PRDM14 | PRDM15 | PRDM16 | PRDM17 |
PRDM2 | PRDM3 | PRDM4 | PRDM5 |
PRDM6 | PRDM7 | PRDM8 | PRDM9 More > |
PREP1 | PRIM2 | PRKAA2 | PRKCA |
PRKCB | PRKCE | PRKDC | PRKRIP1 |
PRMT | prmt1 | PRMT2 | PRMT3 |
PRMT5 | prmt6 | PRMT7 | PRMT8 |
PRMT9 | Progerin | Protamine 2 | Prox1 |
PRPF31 | PRPF40A | PRSS12 | PRSS55 |
PRTFDC1 |
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