Progerin Antibody [J17D10] | Primary Antibodies

Application: Reactivity: Human

Lane 1: HeLa (stably expressing Flag-tagged human Progerin)

Usage Information

Dilution
WB1:10000

Application
WB

Reactivity
Human

Source
Mouse Monoclonal Antibody

Storage Buffer
PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage (from the date of receipt)
-20°C (avoid freeze-thaw cycles), 2 years

Predicted MW Observed MW
74 kDa 70 kDa
^*Why do the predicted and actual molecular weights differ? The following reasons may explain differences between the predicted and actual protein molecular weight.

Datasheet & SDS

Biological Description

Specificity
Progerin Antibody [J17D10] detects endogenous levels of total Progerin protein.

Clone
J17D10

Synonym(s)
LMN1; LMNA; Prelamin-A/C

Background
Progerin (lamin AΔ50) is a pathogenic 607-amino-acid farnesylated truncation mutant of lamin A generated by a cryptic splice site activation in the LMNA gene (c.1824C>T, G608G), which eliminates the ZMPSTE24 cleavage site required for normal lamin A maturation. As a result, progerin retains a C-terminal CaaX (CSIM) farnesylation motif but lacks residues 647–656, preventing defarnesylation and leading to its permanent anchorage at the inner nuclear membrane. Progerin mirrors wild-type lamin A/C in its N-terminal head, central α-helical rod, and Ig-fold domains, but its aberrant farnesylation promotes toxic aggregate formation, clustering of SUN1/emerin, distortion of nuclear pore complexes, and increased lamina rigidity through enhanced F-actin coupling. Progerin disrupts nuclear architecture and genome maintenance by sequestering DNA repair factors (causing persistent 53BP1/γH2AX foci), depleting heterochromatin (H3K9me3 loss, lamina-associated domain relocation), and sustaining DNA damage signaling (ATM/ATR-Chk1/2-p53-p21 mediated senescence). It also impairs Wnt/β-catenin signaling, affects cytoskeletal-nuclear connections (deregulating ERK1/2 via abnormal LINC complexes), and drives cellular exhaustion and loss in vascular smooth muscle, adipocytes, and osteoblasts, manifesting clinically as the premature aging phenotypes of Hutchinson-Gilford Progeria Syndrome (HGPS), including lipodystrophy, osteolysis, and atherosclerosis.

Background

Progerin (lamin AΔ50) is a pathogenic 607-amino-acid farnesylated truncation mutant of lamin A generated by a cryptic splice site activation in the LMNA gene (c.1824C>T, G608G), which eliminates the ZMPSTE24 cleavage site required for normal lamin A maturation. As a result, progerin retains a C-terminal CaaX (CSIM) farnesylation motif but lacks residues 647–656, preventing defarnesylation and leading to its permanent anchorage at the inner nuclear membrane. Progerin mirrors wild-type lamin A/C in its N-terminal head, central α-helical rod, and Ig-fold domains, but its aberrant farnesylation promotes toxic aggregate formation, clustering of SUN1/emerin, distortion of nuclear pore complexes, and increased lamina rigidity through enhanced F-actin coupling. Progerin disrupts nuclear architecture and genome maintenance by sequestering DNA repair factors (causing persistent 53BP1/γH2AX foci), depleting heterochromatin (H3K9me3 loss, lamina-associated domain relocation), and sustaining DNA damage signaling (ATM/ATR-Chk1/2-p53-p21 mediated senescence). It also impairs Wnt/β-catenin signaling, affects cytoskeletal-nuclear connections (deregulating ERK1/2 via abnormal LINC complexes), and drives cellular exhaustion and loss in vascular smooth muscle, adipocytes, and osteoblasts, manifesting clinically as the premature aging phenotypes of Hutchinson-Gilford Progeria Syndrome (HGPS), including lipodystrophy, osteolysis, and atherosclerosis.

References
https://pubmed.ncbi.nlm.nih.gov/19926845/ https://pubmed.ncbi.nlm.nih.gov/32799420/

Reference Table for Selecting PVDF Membrane Pore Size Specifications

Protein Size (kDa)	PVDF Transfer Membrane Pore Size (μm)
< 10	0.22
10-20	0.22
20-30	0.45
30-45	0.45
45-70	0.45
80-100	0.45
100-140	0.45
> 140	0.45

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Protein Size (kDa)	Separating Gel Percentage
4-40	20%
12-45	15%
10-70	12.5%
15-100	10%
25-100	8%
60-210	5%