discontinued

AM50371PU-N Progerin antibody

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0.1 mg / €420.00

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Mouse anti Human Progerin 13A4

AM50371PU-N

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  • AM50371PU-N
  • AM50371PU-N

Product Description for Progerin

Mouse anti Human Progerin 13A4.
Presentation: Purified
Product is tested for Enzyme Immunoassay, Immunocytochemistry/Immunofluorescence, Immunoprecipitation, Western blot / Immunoblot.

Properties for Progerin

Product Category Primary Antibodies
Quantity 0.1 mg
Synonyms CMD1A, EMD2, FPL, FPLD, LDP1, LFP, LMN1, LMNA, LMNC
Presentation Purified
Reactivity Hu
Applications E, ICC/IF, IP, WB
Clonality Monoclonal
Clone 13A4
Host Mouse
Isotype IgG1
Shipping to Worldwide
PDF datasheet View Datasheet
Manufacturer Acris Antibodies GmbH
Material safety datasheet MSDS for Monoclonal Antibodies (de)

Datasheet Extract

Immunogen
Swiss Prot Num:
Q6UYC3
Immunogen:
KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).
Isotype control AM03095PU-N, SM10P (for use in human samples)
Application Immunoprecipitation.
Western Blot.
Immunocytochemistry.
ELISA.
Background 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.
Concentration 1.0 mg/ml (lot-specific)
Storage Store undiluted at 2-8°C.
Shelf life: one year from despatch.
Format
Purification:
Protein G Chromatography
Buffer System:
0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide.
State:
Liquid purified IgG fraction from Ascites
Purified
Specificity
Specificity:
Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
Human. Other species not tested.

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