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Anti-Progerin Antibody, clone 13A4
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Species Reactivity Key Applications Host Format Antibody Type
H WB, ICC, ELISA, IP Mouse Purified Monoclonal Antibody
Description:
Anti-Progerin Antibody, clone 13A4
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Trade Name:
Upstate (Millipore)
Specificity:
Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
Molecular Weight:
70 kDa
Immunogen:
KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).
Clone:
13A4
Isotype:
IgG1κ
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.
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Species Reactivity:
Human
Species Reactivity Note:
Human. Other species not tested.
Control:
HeLa cells expressing human progerin
Quality Assurance:
Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin.
Purification Method:
Protein G purfied
Presentation:
Protein G purified monoclonal antibody from ascites. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide.
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.
UniProt Number:
Q6UYC3
Entrez Gene Number:
NP_005563
Gene Symbol:
LMNA
Alternate Names:
LMNA
CMD1A
LMNC
LMN1
LDP1
LFP
FPLD
FPL
EMD2
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Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Key Applications:
Western Blotting
Immunocytochemistry
ELISA
Immunoprecipitation
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 gird |