05-1231
Anti-Progerin Antibody, clone 13A4
clone 13A4, Upstate®, from mouse
Synonym(s):
LMNA, CMD1A, LMNC, LMN1, LDP1, LFP, FPLD, FPL, EMD2
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All Photos(3)
About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
13A4, monoclonal
species reactivity
human
manufacturer/tradename
Upstate®
technique(s)
ELISA: suitable
immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Gene Information
human ... LMNA(4000)
General description
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.
The previously assigned protein identifier Q6UYC3 has been merged into P02545. Full details can be found on the UniProt database.
Specificity
Other species not tested.
Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
Immunogen
KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).
Application
Research Category
Cell Structure
Cell Structure
This Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin.
Quality
Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin.
Target description
70 kDa
Physical form
Format: Purified
Protein G Purified
Protein G purified immunoglobulin. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide.
Storage and Stability
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.
For maximum recovery of product, centrifuge the vial prior to removing the cap.
Analysis Note
Control
HeLa cells expressing human progerin
HeLa cells expressing human progerin
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
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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Storage Class Code
12 - Non Combustible Liquids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Anahita Mojiri et al.
European heart journal, 42(42), 4352-4369 (2021-08-15)
Hutchinson-Gilford progeria syndrome (HGPS) is an accelerated ageing syndrome associated with premature vascular disease and death due to heart attack and stroke. In HGPS a mutation in lamin A (progerin) alters nuclear morphology and gene expression. Current therapy increases the
Manuela Loi et al.
Oncotarget, 7(13), 15662-15677 (2015-12-25)
Chromatin disorganization is one of the major alterations linked to prelamin A processing impairment. In this study we demonstrate that BAF is necessary to modulate prelamin A effects on chromatin structure. We show that when prelamin A and BAF cannot
Asao Noda et al.
Genes and environment : the official journal of the Japanese Environmental Mutagen Society, 37, 13-13 (2015-01-01)
Progerin, the protein responsible for the Hutchinson-Gilford Progeria Syndrome (HGPS), is a partially deleted form of nuclear lamin A, and its expression has been suggested as a cause for dysfunctional nuclear membrane and premature senescence. To examine the role of
Mzwanele Ngubo et al.
Aging cell, 23(7), e14150-e14150 (2024-04-05)
Hutchinson-Gilford Progeria syndrome (HGPS) is a lethal premature aging disorder caused by a de novo heterozygous mutation that leads to the accumulation of a splicing isoform of Lamin A termed progerin. Progerin expression deregulates the organization of the nuclear lamina
Seul-Ki Park et al.
Experimental dermatology, 26(10), 889-895 (2017-02-14)
Metformin is a popular antidiabetic biguanide, which has been considered as a candidate drug for cancer treatment and ageing prevention. Hutchinson-Gilford progeria syndrome (HGPS) is a devastating disease characterized by premature ageing and severe age-associated complications leading to death. The
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