Anti-Lamin A 抗体 (ab26300)
Key features and details
- Rabbit polyclonal to Lamin A
- Suitable for: ICC/IF, WB
- Knockout validated
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
リコンビナント抗体で、ロット間での高い再現性を実現
- 異なるロット間での安定した再現性
- 容易なスケールアップ
- 評価試験による特異性の確認済み
- 倫理基準に準拠 - アニマル・フリーの生産
製品の概要
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製品名
Anti-Lamin A antibody
Lamin A 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Lamin A -
由来種
Rabbit -
特異性
Replenishment batches of ab26300 are tested in WB. Previous batches were additionally validated in ICC/IF. This application is still expected to work and is covered by our Abpromise guarantee.
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アプリケーション
適用あり: ICC/IF, WBmore details -
種交差性
交差種: Mouse, Rat, Human
交差が予測される動物種: Chicken, Pig -
免疫原
Synthetic peptide conjugated to KLH derived from within residues 550 to the C-terminus of Human Lamin A.
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ポジティブ・コントロール
- ab26300 gave a positive result in the following Whole Cell Lysates A431 NIH 3T3 PC12 ICC-IF: Hela cells
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特記事項
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
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: 98.98% PBS, 1% BSA
Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help. -
Concentration information loading...
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精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
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ab26300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 |
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ICC/IF | (4) |
Use a concentration of 1 µg/ml.
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WB | (14) |
Use a concentration of 1 µg/ml. Detects a band of approximately 76 kDa (predicted molecular weight: 74 kDa).
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特記事項 |
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ICC/IF
Use a concentration of 1 µg/ml. |
WB
Use a concentration of 1 µg/ml. Detects a band of approximately 76 kDa (predicted molecular weight: 74 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. Play an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics.
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 celle (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
Defects in LMNA are the cause of Emery-Dreifuss muscular dystrophy type 2 (EDMD2) [MIM:181350]. A degenerative myopathy characterized by weakness and atrophy of muscle without involvement of the nervous system, early contractures of the elbows, Achilles tendons and spine, and cardiomyopathy associated with cardiac conduction defects.
Defects in LMNA are the cause of cardiomyopathy dilated type 1A (CMD1A) [MIM:115200]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in LMNA are the cause of familial partial lipodystrophy type 2 (FPLD2) [MIM:151660]; also known as familial partial lipodystrophy Dunnigan type. A disorder characterized by the loss of subcutaneous adipose tissue in the lower parts of the body (limbs, buttocks, trunk). It is accompanied by an accumulation of adipose tissue in the face and neck causing a double chin, fat neck, or cushingoid appearance. Adipose tissue may also accumulate in the axillae, back, labia majora, and intraabdominal region. Affected patients are insulin-resistant and may develop glucose intolerance and diabetes mellitus after age 20 years, hypertriglyceridemia, and low levels of high density lipoprotein cholesterol.
Defects in LMNA are the cause of limb-girdle muscular dystrophy type 1B (LGMD1B) [MIM:159001]. LGMD1B is an autosomal dominant degenerative myopathy with age-related atrioventricular cardiac conduction disturbances, dilated cardiomyopathy, and the absence of early contractures. LGMD1B is characterized by slowly progressive skeletal muscle weakness of the hip and shoulder girdles. Muscle biopsy shows mild dystrophic changes.
Defects in LMNA are the cause of Charcot-Marie-Tooth disease type 2B1 (CMT2B1) [MIM:605588]. CMT2B1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2B1 inheritance is autosomal recessive.
Defects in LMNA are the cause of Hutchinson-Gilford progeria syndrome (HGPS) [MIM:176670]. HGPS is a rare genetic disorder characterized by features reminiscent of marked premature aging. Note=HGPS is caused by the toxic accumulation of a mutant form of lamin-A/C. This mutant protein, called progerin, acts to deregulate mitosis and DNA damage signaling, leading to premature cell death and senescence. Progerin lacks the conserved ZMPSTE24/FACE1 cleavage site and therefore remains permanently farnesylated. Thus, although it can enter the nucleus and associate with the nuclear envelope, it cannot incorporate normally into the nuclear lamina.
Defects in LMNA are the cause of cardiomyopathy dilated with hypergonadotropic hypogonadism (CMDHH) [MIM:212112]. A disorder characterized by the association of genital anomalies, hypergonadotropic hypogonadism and dilated cardiomyopathy. Patients can present other variable clinical manifestations including mental retardation, skeletal anomalies, scleroderma-like skin, graying and thinning of hair, osteoporosis. Dilated cardiomyopathy is characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia.
Defects in LMNA are the cause of mandibuloacral dysplasia with type A lipodystrophy (MADA) [MIM:248370]. A disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, progeroide appearance, partial alopecia, soft tissue calcinosis, joint contractures, and partial lipodystrophy with loss of subcutaneous fat from the extremities. Adipose tissue in the face, neck and trunk is normal or increased.
Defects in LMNA are a cause of lethal tight skin contracture syndrome (LTSCS) [MIM:275210]; also known as restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.
Defects in LMNA are the cause of heart-hand syndrome Slovenian type (HHS-Slovenian) [MIM:610140]. Heart-hand syndrome (HHS) is a clinically and genetically heterogeneous disorder characterized by the co-occurrence of a congenital cardiac disease and limb malformations.
Defects in LMNA are the cause of muscular dystrophy congenital LMNA-related (CMD-LMNA) [MIM:613205]. It is a form of congenital muscular dystrophy. Patients present at birth, or within the first few months of life, with hypotonia, muscle weakness and often with joint contractures. -
配列類似性
Belongs to the intermediate filament family. -
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. Nucleus envelope. 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: 396224 Chicken
- Entrez Gene: 4000 Human
- Entrez Gene: 16905 Mouse
- Entrez Gene: 100126859 Pig
- Entrez Gene: 60374 Rat
- Omim: 150330 Human
- SwissProt: P13648 Chicken
- SwissProt: P02545 Human
see all -
別名
- 70 kDa lamin antibody
- CDDC antibody
- EMD2 antibody
see all
画像
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ab26300 staining Lamin A in HeLa cells. The cells were fixed with 100% methanol (5 min), permeabilized with 0.1% PBS-Triton X-100 for 5 minutes and then blocked with 1% BSA/10% normal goat serum/0.3M glycine in 0.1%PBS-Tween for 1h. The cells were then incubated overnight at 4°C with ab26300 at 5µg/ml and ab7291, Mouse monoclonal [DM1A] to alpha Tubulin - Loading Control. Cells were then incubated with ab150081, Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (Alexa Fluor® 488), pre-adsorbed at 1/1000 dilution (shown in green) and ab150120, Goat polyclonal Secondary Antibody to Mouse IgG - H&L (Alexa Fluor® 594), pre-adsorbed at 1/1000 dilution (shown in pseudocolour magenta). Nuclear DNA was labelled with DAPI (shown in blue). Also suitable in cells fixed with 4% paraformaldehyde (10 min).Image was acquired with a high-content analyser (Operetta CLS, Perkin Elmer) and a maximum intensity projection of confocal sections is shown.
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Lane 1: Wild-type HAP1 cell lysate (20 µg)
Lane 2: Lamin A knockout HAP1 cell lysate (20 µg)
Lane 3: A431 cell lysate (20 µg)
Lane 4: NIH3T3 cell lysate (20 µg)
Lanes 1 - 4: Merged signal (red and green). Green - ab26300 observed at 76 kDa. Red - loading control, ab8245, observed at 37 kDa.
ab26300 was shown to recognize Lamin A in wild-type HAP1 cells along with additional cross-reactive bands. No band was observed when Lamin A knockout samples were examined. Wild-type and Lamin A knockout samples were subjected to SDS-PAGE. ab26300 1ug/ml and ab8245 (loading control to GAPDH) at a dilution of 1/1000 were incubated overnight at 4°C. Blots were developed with Goat anti-Rabbit IgG H&L (IRDye® 800CW) preadsorbed (ab216773) and Goat anti-Mouse IgG H&L (IRDye® 680RD) preadsorbed (ab216776) secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging. -
Anti-Lamin A antibody (ab26300) at 1/1000 dilution + HeLa whole cell extract at 100 µg
Secondary
Goat anti-Rabbit IgG (H+L) HRP Conjugate at 1/10000 dilution
Developed using the ECL technique.
Predicted band size: 74 kDa
Observed band size: 76 kDa why is the actual band size different from the predicted?
Exposure time: 15 secondsBlocking: 5% milk for 30 minutes at 22°C
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All lanes : Anti-Lamin A antibody (ab26300) at 1/1000 dilution
Lane 1 : Mouse NIH-3T3 cells - cytosolic fraction
Lane 2 : Mouse NIH-3T3 cells - nuclear fraction
Lysates/proteins at 25 µg per lane.
Secondary
All lanes : HRP conjugated Goat anti-rabbit at 1/5000 dilution
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 74 kDa
Observed band size: 76 kDa why is the actual band size different from the predicted?
Exposure time: 10 seconds
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Anti-Lamin A antibody (ab26300) at 1 µg/ml + A-431 whole cell lysate (ab7909) at 20 µg
Secondary
IRDye 680 Conjugated Goat Anti-Rabbit IgG (H+L) at 1/15000 dilution
Performed under reducing conditions.
Predicted band size: 74 kDa
Observed band size: 76 kDa why is the actual band size different from the predicted?
Additional bands at: 68 kDa (possible degradation product) -
All lanes : Anti-Lamin A antibody (ab26300) at 1 µg/ml
Lane 1 :Recombinant Human Lamin A protein (ab83472) at 0.1 µg
Lane 2 :Recombinant Human Lamin A protein (ab83472) at 0.01 µg
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 74 kDa
Exposure time: 10 seconds -
All lanes : Anti-Lamin A antibody (ab26300) at 1 µg/ml
Lane 1 : NIH/3T3 whole cell lysate (ab7179)
Lane 2 : PC12 (Rat adrenal pheochromocytoma cell line) Whole Cell Lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : IRDye 680 Conjugated Goat Anti-Rabbit IgG (H+L) at 1/10000 dilution
Performed under reducing conditions.
Predicted band size: 74 kDa
Observed band size: 74 kDa
Additional bands at: 100 kDa, 45 kDa, 70 kDa (possible degradation product). We are unsure as to the identity of these extra bands.
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 (117)
ab26300 は 117 報の論文で使用されています。
- Todorovski V et al. Confined environments induce polarized paraspeckle condensates. Commun Biol 6:145 (2023). PubMed: 36737664
- Pan Q et al. Organic Anion Transporting Polypeptide (OATP) 1B3 is a Significant Transporter for Hepatic Uptake of Conjugated Bile Acids in Humans. Cell Mol Gastroenterol Hepatol 16:223-242 (2023). PubMed: 37146714
- Ianiro G et al. Iron Saturation Drives Lactoferrin Effects on Oxidative Stress and Neurotoxicity Induced by HIV-1 Tat. Int J Mol Sci 24:N/A (2023). PubMed: 37175651
- Skory RM et al. The nuclear lamina couples mechanical forces to cell fate in the preimplantation embryo via actin organization. Nat Commun 14:3101 (2023). PubMed: 37248263
- Schena E et al. Mineralocorticoid Receptor Antagonism Prevents Type 2 Familial Partial Lipodystrophy Brown Adipocyte Dysfunction. Cells 12:N/A (2023). PubMed: 37998321