7188552

Progerin Monoclonal Antibody (13A4), Active Motif™

Mouse Monoclonal Antibody

Manufacturer: Fischer Scientific

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Antigen

Progerin

Concentration

1 mg/mL

Classification

Monoclonal

Form

Liquid

Regulatory Status

RUO

Formulation

70mM tris with 0.07mM EDTA, 105mM NaCl, 30% glycerol, 31mM glycine and 0.035% sodium azide; pH 8

Gene Alias

70 kDa lamin; CDCD1; CDDC; CMD1A; CMT2B1; EMD2; FPL; FPLD; FPLD2; HGPS; IDC; lamin; lamin A/C; lamin A/C-like 1; Lamin AC; Lamin-A/C; LDP1; LFP; LGMD1B; LMN1; LMNA; LMNC; LMNL1; Mutant lamin A/C; Prelamin-A/C; PRO1; progerin mutant; Renal carcinoma antigen NY-REN-32

Gene Symbols

LMNA

Isotype

IgG1 κ

Purification Method

Protein G

Product Type

Antibody

Clone

13A4

Applications

Immunocytochemistry, Immunofluorescence, Western Blot

Conjugate

Unconjugated

Host Species

Mouse

Target Species

Human

Gene Accession No.

P02545

Gene ID (Entrez)

4000

Immunogen

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

Primary or Secondary

Primary

Content And Storage

-20°C, Avoid Freeze/Thaw Cycles

Gene

LMNA

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Description

  • Progerin is a 614 amino acid protein involved in Hutchinson-Gilford progeria syndrome
  • Progerin is most often generated by a point mutation (C1824T) in the LMNA gene that codes for lamin-A/C
  • This mutation activates a cryptic splice site and gives rise to a form of lamin A with a 50-amino acid internal deletion within the C-terminal domain of the protein
  • Approximately 80% of Hutchinson-Gilford progeria syndrome cases carry a single copy of the most common mutation, a silent point mutation, G608G (GGC > GGT), within exon 11 of the LMNA gene.