AB66587

Anti-Progerin antibody [13A4]

Name: Anti-Progerin antibody [13A4] (ab66587) | Abcam
Brand: Abcam Limited
SKU: AB66587
Price: 195 USD
Availability: InStock
Rating: 3 (2 reviews)

(2 Reviews)

(22 Publications )

Mouse Monoclonal Lamin-A/C antibody. Suitable for WB and reacts with Human samples. Cited in 22 publications.

2 Images

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Western blot - Anti-Progerin antibody [13A4] (AB66587)

Incubation with primary antibody : 2 hours at RT, 0.5% NFDM in PBS-Tween.

Lane 1:

Anti Lamin A/C 3A6-4C11 at 1/1000 dilution

Lanes 2 and 5:

Western blot - Anti-Progerin antibody [13A4] (ab66587) at 1/200 dilution

Lanes 3 and 6:

Western blot - Anti-Progerin antibody [13A4] (ab66587) at 1/500 dilution

Lanes 4 and 7:

Western blot - Anti-Progerin antibody [13A4] (ab66587) at 1/1000 dilution

All lanes:

HeLa cells stably expressing Flag-tagged human Progerin

Predicted band size: 74 kDa

Observed band size: 70 kDa

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Exposure time: 2min

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Western blot - Anti-Progerin antibody [13A4] (AB66587)

ab66587 specifically detecting human Progerin by Western blotting.

Western blot analysis (10% PAGE) of whole cell lysate of HeLa cells ectopically expressing Flag-tagged human proteins (protein accession number AAR29466). The membrane was cut into strips and each strip was incubated separately with the following antibodies :

Lane 1:

anti Lamin A/C antibody at 1/2000 dilution

Lane 2:

anti Flag-tag antibody at 1/5000 dilution

Lane 3:

Western blot - Anti-Progerin antibody [13A4] (ab66587) at 1/500 dilution

All lanes:

Whole cell lysate of HeLa cells ectopically expressing Flag-tagged human proteins (protein accession number AAR29466)

Predicted band size: 74 kDa

false

Key facts

Host species

Mouse

Clonality

Monoclonal

Clone number

13A4

Isotype

IgG1

Light chain type

kappa

Carrier free

Reacts with

Human

Applications

applications

Immunogen

The exact immunogen used to generate this antibody is proprietary information.

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000", "WB-species-notes": "<p></p>" } } }

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Product details

Progerin is expressed as the result of a de novo point mutation in the lamin A gene and is the underlying cause of Hutchison-Gilford progeria syndrome (accelerated aging). There is now also evidence that small amounts of Progerin are also produced in normal cells and that this might have a link with the normal aging process (Cao et al., PNAS, 2007 Mar 20;104(12):4949-54.). Gly608Gly is the most frequent HGPS-associated mutation. It is a silent base substitution that activates a cryptic splice donor in exon 11 of LMNA (BOX 3). Use of this anomalous splice donor leads to the loss of 150 nucleotides from the 3' end of exon 11 in the mature lamin A mRNA, and internal deletion of 50 amino-acid residues from the C terminus of lamin A. Progerin is the resulting mutant protein. Progerin retains its C-terminal CAAX motif, and therefore is farnesylated.

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification

Storage buffer

pH: 7.4 Preservative: 0.02% Sodium azide Constituents: PBS, 6.97% L-Arginine

Shipped at conditions

Blue Ice

Appropriate short-term storage duration

1-2 weeks

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

-20°C

Aliquoting information

Upon delivery aliquot

Storage information

Avoid freeze / thaw cycle

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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

Progerin also known as a truncated version of Lamin A (LMNA) is a mutant protein that emerges due to a cryptic splice site in the LMNA gene. This results in a 607 amino acid protein with a mass of approximately 65 kDa. Progerin is expressed in the cell nucleus and accumulates over time predominantly affecting cells with high turnover rates such as those in the skin cardiovascular system and adipose tissues. This abnormal protein disrupts the normal function of the nuclear lamina.

Biological function summary

Progerin acts by compromising cellular structural integrity and altering gene expression. It integrates into the nuclear lamina but fails to undergo proper post-translational modification which is vital for cellular functions like mitosis nuclear migration and chromatin organization. Progerin's presence affects the mechanical properties of the nucleus hindering its role in stress response and inducing cellular aging. This mutant protein functions independently as it does not form part of a larger protein complex but rather disturbs the assembly of the lamina network.

Pathways

Progerin influences the mechanistic pathways responsible for cellular aging and apoptosis. It disrupts the Wnt/β-catenin pathway affecting cell cycle regulation and the MAPK signaling pathway which relates to stress and growth responses. Progerin shares a close association with prelamin A and Lamin C as all these proteins derive from the same LMNA gene and interact through shared pathways influencing nuclear shape and transcriptome stability.

Progerin plays a significant role in Hutchinson-Gilford Progeria Syndrome (HGPS) and is implicated in age-related disorders like arteriosclerosis. HGPS results from the accumulation of Progerin leading to premature aging due to the faulty nuclear architecture. Progerin's relation with other proteins such as Lamin B1 and Lamin C affects the nuclear envelope's integrity exacerbating the progression of these disorders. The presence of Progerin may serve as a potential target for therapeutic interventions aimed at blocking its production or function which could mitigate its effects on aging-related pathologies.

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:

Visit the General protocols
Visit the Troubleshooting

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Target data

Lamin-A/C. Lamins are intermediate filament proteins that assemble into a filamentous meshwork, and which constitute the major components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane (PubMed : 10080180, PubMed : 10580070, PubMed : 10587585, PubMed : 10814726, PubMed : 11799477, PubMed : 12075506, PubMed : 12927431, PubMed : 15317753, PubMed : 18551513, PubMed : 18611980, PubMed : 2188730, PubMed : 22431096, PubMed : 2344612, PubMed : 23666920, PubMed : 24741066, PubMed : 31434876, PubMed : 31548606, PubMed : 37788673, PubMed : 37832547). Lamins provide a framework for the nuclear envelope, bridging the nuclear envelope and chromatin, thereby playing an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics (PubMed : 10080180, PubMed : 10580070, PubMed : 10587585, PubMed : 10814726, PubMed : 11799477, PubMed : 12075506, PubMed : 12927431, PubMed : 15317753, PubMed : 18551513, PubMed : 18611980, PubMed : 22431096, PubMed : 23666920, PubMed : 24741066, PubMed : 31548606, PubMed : 37788673, PubMed : 37832547). Lamin A and C also regulate matrix stiffness by conferring nuclear mechanical properties (PubMed : 23990565, PubMed : 25127216). The structural integrity of the lamina is strictly controlled by the cell cycle, as seen by the disintegration and formation of the nuclear envelope in prophase and telophase, respectively (PubMed : 2188730, PubMed : 2344612). Lamin A and C are present in equal amounts in the lamina of mammals (PubMed : 10080180, PubMed : 10580070, PubMed : 10587585, PubMed : 10814726, PubMed : 11799477, PubMed : 12075506, PubMed : 12927431, PubMed : 15317753, PubMed : 18551513, PubMed : 18611980, PubMed : 22431096, PubMed : 23666920, PubMed : 31548606). Also involved in DNA repair : recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends (PubMed : 31548606). Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation (PubMed : 10080180, PubMed : 10814726, PubMed : 11799477, PubMed : 18551513, PubMed : 22431096). Required for osteoblastogenesis and bone formation (PubMed : 12075506, PubMed : 15317753, PubMed : 18611980). Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone (PubMed : 10587585). Required for cardiac homeostasis (PubMed : 10580070, PubMed : 12927431, PubMed : 18611980, PubMed : 23666920).. Prelamin-A/C. Prelamin-A/C can accelerate smooth muscle cell senescence (PubMed : 20458013). It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence (PubMed : 20458013).

See full target information LMNA

Alternative Names

LMN1, LMNA, Prelamin-A/C

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Publications (22)

Recent publications for all applications. Explore the full listand refine your search

Scientific reports 14:19703 PubMed39181932

2024

Premature aging effects on COVID-19 pathogenesis: new insights from mouse models.

Applications

Unspecified application

Species

Unspecified reactive species

Wu Haoyu,Liu Meiqin,Sun Jiaoyang,Hong Guangliang,Lin Haofeng,Chen Pan,Quan Xiongzhi,Wu Kaixin,Hu Mingli,Yang Xuejie,Ingo Lämmermann,Johannes Grillari,Shi Zhengli,Chen Jiekai,Wu Guangming

Aging cell 23:e14303 PubMed39113346

2024

The activation of cGAS-STING pathway causes abnormal uterine receptivity in aged mice.

Applications

Unspecified application

Species

Unspecified reactive species

Si-Ting Chen,Wen-Wen Shi,Feng Ran,Cheng-Kan Liu,Hui-Na Luo,Li-Juan Wu,Ying Wu,Tong-Tong Zhang,Zeng-Ming Yang

Aging cell 23:e14143 PubMed38482753

2024

Mitophagy defect mediates the aging-associated hallmarks in Hutchinson-Gilford progeria syndrome.

Applications

Unspecified application

Species

Unspecified reactive species

Yingying Sun,Le Xu,Yi Li,Shunze Jia,Gang Wang,Xufeng Cen,Yuyan Xu,Zhongkai Cao,Jingjing Wang,Ning Shen,Lidan Hu,Jin Zhang,Jianhua Mao,Hongguang Xia,Zhihong Liu,Xudong Fu

Nature aging 3:185-201 PubMed37118121

2023

Unique progerin C-terminal peptide ameliorates Hutchinson-Gilford progeria syndrome phenotype by rescuing BUBR1.

Applications

Unspecified application

Species

Unspecified reactive species

Na Zhang,Qianying Hu,Tingting Sui,Lu Fu,Xinglin Zhang,Yu Wang,Xiaojuan Zhu,Baiqu Huang,Jun Lu,Zhanjun Li,Yu Zhang

The EMBO journal 42:e110937 PubMed36382717

2022

Anti-hsa-miR-59 alleviates premature senescence associated with Hutchinson-Gilford progeria syndrome in mice.

Applications

Unspecified application

Species

Unspecified reactive species

Qianying Hu,Na Zhang,Tingting Sui,Guanlin Li,Zhiyao Wang,Mingyue Liu,Xiaojuan Zhu,Baiqu Huang,Jun Lu,Zhanjun Li,Yu Zhang

Nucleic acids research 50:9948-9965 PubMed36099415

2022

Lamin A/C impairments cause mitochondrial dysfunction by attenuating PGC1α and the NAMPT-NAD+ pathway.

Applications

Unspecified application

Species

Unspecified reactive species

Scott Maynard,Arnaldur Hall,Panagiotis Galanos,Salvatore Rizza,Tatsuro Yamamoto,Helena Hagner Gram,Sebastian H N Munk,Muhammad Shoaib,Claus Storgaard Sørensen,Vilhelm A Bohr,Mads Lerdrup,Apolinar Maya-Mendoza,Jiri Bartek

Life science alliance 5: PubMed36104080

2022

Mesenchymal stem cells derived from patients with premature aging syndromes display hallmarks of physiological aging.

Applications

Unspecified application

Species

Unspecified reactive species

Jean Philippe Trani,Raphaël Chevalier,Leslie Caron,Claire El Yazidi,Natacha Broucqsault,Léa Toury,Morgane Thomas,Karima Annab,Bernard Binetruy,Annachiara De Sandre-Giovannoli,Nicolas Levy,Frédérique Magdinier,Jérôme D Robin

European heart journal open 2:oeac047 PubMed36117952

2022

Vascular senescence in progeria: role of endothelial dysfunction.

Applications

Unspecified application

Species

Unspecified reactive species

Qiu Xu,Anahita Mojiri,Luay Boulahouache,Elisa Morales,Brandon K Walther,John P Cooke

Scientific reports 11:9122 PubMed33907225

2021

Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A.

Applications

Unspecified application

Species

Unspecified reactive species

So-Mi Kang,Min-Ho Yoon,Su-Jin Lee,Jinsook Ahn,Sang Ah Yi,Ki Hong Nam,Soyoung Park,Tae-Gyun Woo,Jung-Hyun Cho,Jaecheol Lee,Nam-Chul Ha,Bum-Joon Park

Communications biology 4:5 PubMed33398110

2021

Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome.

Applications

Unspecified application

Species

Unspecified reactive species

So-Mi Kang,Min-Ho Yoon,Jinsook Ahn,Ji-Eun Kim,So Young Kim,Seock Yong Kang,Jeongmin Joo,Soyoung Park,Jung-Hyun Cho,Tae-Gyun Woo,Ah-Young Oh,Kyu Jin Chung,So Yon An,Tae Sung Hwang,Soo Yong Lee,Jeong-Su Kim,Nam-Chul Ha,Gyu-Yong Song,Bum-Joon Park

View all publications

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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.

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Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

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Anti-Progerin antibody [13A4]

Western blot - Anti-Progerin antibody [13A4] (AB66587)

Western blot - Anti-Progerin antibody [13A4] (AB66587)

Key facts

Host species

Clonality

Clone number

Isotype

Light chain type

Carrier free

Reacts with

Applications

Immunogen

Complementary Products

Primary Antibodies

Primary Antibodies

Primary Antibodies

Primary Antibodies

Reactivity data

Product details

Properties and storage information

Form

Purification technique

Storage buffer

Shipped at conditions

Appropriate short-term storage duration

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Aliquoting information

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Alternative Names

Publications (22)

Product promise