Progerin antibody (mAb)

Clone: 13A4
Catalog No: 39965 Format: 100 µg $435 Buy
Catalog No: 39966 Format: 10 µg $105 Buy

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Antibody Type:
Monoclonal
Isotype:
IgG1k
Purification:
Protein G Chromatography
Host:
Mouse
Molecular Weight:
69 kDa
Reactivity:
Human

Applications

Western Blot Validated Immunofluorescence Validated Immunocytochemistry Validated

Application Notes


Published Applications:
ICC/IF
WB
See references for more information. Individual optimization may be required.

Immunogen

This Progerin antibody was raised against a peptide corresponding to amino acids 604-611 of human Progerin.

Buffer

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.

References

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.

Compatible Secondary Antibodies

Active Motif has developed a variety of high-quality secondary antibodies for most applications, with anti-rabbit and anti-mouse conjugates to a broad line of high-quality fluorescent molecules, as well as horse radish peroxidase (HRP). Visit our Secondary Antibody Conjugates page to find the perfect secondaries for immunofluorescence experiments with Progerin antibody (mAb).

Storage

Some products may be shipped at room temperature. This will not affect their stability or performance. Upon receipt, unconjugated antibodies may be stored at -20°C for up to 2 years. Fluorophore- & enzyme-conjugated antibodies should be stored at 4°C. Fluorophore-conjugated antibodies should be protected from light. Keep reagents on ice when not in storage; to avoid repeated freeze/thaw cycles, we recommend aliquoting items that will be stored frozen into single-use fractions prior to freezing.

Guarantee

This product is guaranteed for 6 months from date of receipt.

This product is for research use only and is not for use in diagnostic procedures.

Application Key

  • ChIP = Chromatin Immunoprecipitation;
  • DB = Dot Blot;
  • ELISA = Enzyme-linked Immunosorbent Assay;
  • EMSA = Electrophoretic Mobility Shift Assay
  • FC = Flow Cytometry;
  • ICC = Immunocytochemistry;
  • IF = Immunofluorescence;
  • IHC = Immunohistochemistry;
  • IP = Immunoprecipitation;
  • MeDIP = Methyl-DNA Immunoprecipitation;
  • TR-FRET = Time-Resolved Fluorescence Resonance Energy Transfer;
  • WB = Western Blotting