Anti-Progerin 抗体 [13A4] (ab66587)
![Western blot - Anti-Progerin antibody [13A4] (ab66587)](https://www.abcam.co.jp/ps/products/66/ab66587/Images/ab66587-80463-anti-progerin-antibody-13a4-western-blot.jpg "Western blot - Anti-Progerin antibody [13A4] (ab66587)")
Key features and details
- Mouse monoclonal [13A4] to Progerin
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG1
製品の概要
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製品名
Anti-Progerin antibody [13A4]
Progerin 一次抗体 製品一覧 -
製品の詳細
Mouse monoclonal [13A4] to Progerin -
由来種
Mouse -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Human -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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ポジティブ･コントロール
- HeLa Flag-Progerin or primary progeria fibroblasts.
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特記事項
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.
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com.
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製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
バッファー
pH: 7.40
Preservative: 0.02% Sodium azide
Constituents: PBS, 6.97% L-Arginine -
Concentration information loading... -
精製度
Protein G purified -
一次抗体 備考
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. -
ポリ/モノ
モノクローナル -
クローン名
13A4 -
ミエローマ
x63-Ag8.653 -
アイソタイプ
IgG1 -
軽鎖の種類
kappa -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab66587の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| WB | (1) |
1/1000. Detects a band of approximately 70 kDa.
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| 特記事項 |
|---|
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WB
1/1000. Detects a band of approximately 70 kDa. |
ターゲット情報
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機能
Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone.
Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence. -
組織特異性
In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress. -
関連疾患
Emery-Dreifuss muscular dystrophy 2, autosomal dominant
Emery-Dreifuss muscular dystrophy 3, autosomal recessive
Cardiomyopathy, dilated 1A
Lipodystrophy, familial partial, 2
Limb-girdle muscular dystrophy 1B
Charcot-Marie-Tooth disease 2B1
Hutchinson-Gilford progeria syndrome
Cardiomyopathy, dilated, with hypergonadotropic hypogonadism
Mandibuloacral dysplasia with type A lipodystrophy
Lethal tight skin contracture syndrome
Heart-hand syndrome Slovenian type
Muscular dystrophy congenital LMNA-related
Defects in LMNA may cause a late-onset cardiocutaneous progeria syndrome characterized by cutaneous manifestations of aging appearing in the third decade of life, cardiac valve calcification and dysfunction, prominent atherosclerosis, and cardiomyopathy, leading to death on average in the fourth decade. -
配列類似性
Belongs to the intermediate filament family.
Contains 1 LTD domain. -
翻訳後修飾
Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations.
Proteolytic cleavage of the C-terminal of 18 residues of prelamin-A/C results in the production of lamin-A/C. The prelamin-A/C maturation pathway includes farnesylation of CAAX motif, ZMPSTE24/FACE1 mediated cleavage of the last three amino acids, methylation of the C-terminal cysteine and endoproteolytic removal of the last 15 C-terminal amino acids. Proteolytic cleavage requires prior farnesylation and methylation, and absence of these blocks cleavage.
Sumoylation is necessary for the localization to the nuclear envelope.
Farnesylation of prelamin-A/C facilitates nuclear envelope targeting. -
細胞内局在
Nucleus speckle and Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin-A/C, which can then be inserted into the nuclear lamina. EMD is required for proper localization of non-farnesylated prelamin-A/C. - Information by UniProt
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参照データベース
- Entrez Gene: 4000 Human
- Omim: 150330 Human
- SwissProt: P02545 Human
- SwissProt: Q6UYC3 Human
- Unigene: 594444 Human
- Unigene: 706897 Human
- Unigene: 733671 Human
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別名
- 70 kDa lamin antibody
- CDCD1 antibody
- CDDC antibody
see all
画像
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Western blot - Anti-Progerin antibody [13A4] (ab66587)Lane 1 : anti Lamin A/C antibody at 1/2000 dilution
Lane 2 : anti Flag-tag antibody at 1/5000 dilution
Lane 3 : 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)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:
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![Western blot - Anti-Progerin antibody [13A4] (ab66587)](https://www.abcam.co.jp/ps/products/66/ab66587/Images/ab66587-80462-anti-progerin-antibody-13a4-western-blot.jpg)
Western blot - Anti-Progerin antibody [13A4] (ab66587)Lane 1 : Anti Lamin A/C 3A6-4C11 at 1/1000 dilution
Lanes 2 & 5 : Anti-Progerin antibody [13A4] (ab66587) at 1/200 dilution
Lanes 3 & 6 : Anti-Progerin antibody [13A4] (ab66587) at 1/500 dilution
Lanes 4 & 7 : Anti-Progerin antibody [13A4] (ab66587) at 1/1000 dilution
All lanes : HeLa cells stably expressing Flag-tagged human Progerin
Observed band size: 70 kDa why is the actual band size different from the predicted?
Exposure time: 2 minutes
Incubation with primary antibody: 2 hours at RT, 0.5% NFDM in PBS-Tween.
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (19)
ab66587 は 19 報の論文で使用されています。
- Zhang N et al. Unique progerin C-terminal peptide ameliorates Hutchinson-Gilford progeria syndrome phenotype by rescuing BUBR1. Nat Aging 3:185-201 (2023). PubMed: 37118121
- Hu Q et al. Anti-hsa-miR-59 alleviates premature senescence associated with Hutchinson-Gilford progeria syndrome in mice. EMBO J 42:e110937 (2023). PubMed: 36382717
- Maynard S et al. Lamin A/C impairments cause mitochondrial dysfunction by attenuating PGC1α and the NAMPT-NAD+ pathway. Nucleic Acids Res 50:9948-9965 (2022). PubMed: 36099415
- Trani JP et al. Mesenchymal stem cells derived from patients with premature aging syndromes display hallmarks of physiological aging. Life Sci Alliance 5:N/A (2022). PubMed: 36104080
- Xu Q et al. Vascular senescence in progeria: role of endothelial dysfunction. Eur Heart J Open 2:oeac047 (2022). PubMed: 36117952