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Tags & Cell Markers Subcellular Markers Nucleus Nuclear Envelope

Anti-Lamin A抗体(ab2559)

  • Datasheet
  • SDS
Reviews (1)Q&A (4)References (7)

Product price, shipping and contact information

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Key features and details

  • Rabbit polyclonal to Lamin A
  • Reacts with: Mouse, Rat, Human
  • Isotype: IgG

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概述

  • 产品名称

    Anti-Lamin A抗体
    参阅全部 Lamin A 一抗
  • 描述

    兔多克隆抗体to Lamin A
  • 宿主

    Rabbit
  • 特异性

    This antibody recognizes full length (70 kDa) and small fragment (28 kDa) of Lamin A. This antibody will recognise lamanin C in addition to lamanin A.
  • 种属反应性

    与反应: Mouse, Rat, Human
  • 免疫原

    Synthetic peptide:

    ETKR RHETRLVEID

    , corresponding to amino acids 217-230 of Human Lamin A
    Run BLAST with BLAST the sequence with ExPASy Run BLAST with BLAST the sequence with NCBI
  • 常规说明

    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 the problem with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation.

    One factor contributing to the crisis is the use of antibodies that are not suitable. This can lead to misleading results and the use of incorrect data informing project assumptions and direction. To help address this challenge, we have introduced an application and species grid on our primary antibody datasheets to make it easy to simplify identification of the right antibody for your needs.

    Learn more here.

性能

  • 形式

    Liquid
  • 存放说明

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液

    pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: 50% Glycerol, 1% BSA
  • Concentration information loading...
  • 纯度

    Immunogen affinity purified
  • 克隆

    多克隆
  • 同种型

    IgG
  • 研究领域

    • Tags & Cell Markers
    • Subcellular Markers
    • Nucleus
    • Nuclear Envelope
    • Signal Transduction
    • Cytoskeleton / ECM
    • Cytoskeleton
    • Intermediate Filaments
    • Class V
    • Lamins

相关产品

  • Compatible Secondaries

    • Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077)
    • Goat Anti-Rabbit IgG H&L (HRP) (ab205718)
  • Isotype control

    • Rabbit IgG, polyclonal - Isotype Control (ChIP Grade) (ab171870)
  • Recombinant Protein

    • Recombinant Human Lamin A protein (ab83472)

靶标

  • 功能

    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.
  • 组织特异性

    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.
  • 疾病相关

    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.
  • 序列相似性

    Belongs to the intermediate filament family.
  • 翻译后修饰

    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.
  • 细胞定位

    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.
  • Target information above from: UniProt accession P02545 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • 数据库链接

    • Entrez Gene: 4000 Human
    • Entrez Gene: 16905 Mouse
    • Entrez Gene: 60374 Rat
    • Omim: 150330 Human
    • SwissProt: P02545 Human
    • SwissProt: P48678 Mouse
    • SwissProt: P48679 Rat
    • Unigene: 594444 Human
    • Unigene: 243014 Mouse
    • Unigene: 471227 Mouse
    • Unigene: 44161 Rat
    see all
  • 别名

    • 70 kDa lamin antibody
    • CDDC antibody
    • EMD2 antibody
    • FPL antibody
    • FPLD antibody
    • HGPS antibody
    • IDC antibody
    • LAMIN A antibody
    • lamin A/C antibody
    • LAMIN C antibody
    • Lamin-A/C antibody
    • LDP1 antibody
    • LFP antibody
    • LMN 1 antibody
    • LMN A antibody
    • LMN C antibody
    • LMNA antibody
    • LMNA_HUMAN antibody
    • LMNC antibody
    • PRO1 antibody
    • Renal carcinoma antigen NY-REN-32 antibody
    see all

实验方案

  • Western blot protocols

Click here to view the general protocols

数据表及文件

  • SDS download

  • Datasheet download

    Download

文献 (7)

发表研究结果有使用 ab2559?请让我们知道,以便我们可以引用本数据表中的参考文章。

ab2559 被引用在 7 文献中.

  • Papac-Milicevic N  et al. The interferon stimulated gene 12 inactivates vasculoprotective functions of NR4A nuclear receptors. Circ Res 110:e50-63 (2012). PubMed: 22427340
  • Ando Y  et al. Nuclear pore complex protein mediated nuclear localization of dicer protein in human cells. PLoS One 6:e23385 (2011). WB ; Human . PubMed: 21858095
  • Végran F  et al. A short caspase-3 isoform inhibits chemotherapy-induced apoptosis by blocking apoptosome assembly. PLoS One 6:e29058 (2011). WB . PubMed: 22216167
  • Ishibashi M  et al. Reduced VLDL clearance in Apoe(-/-)Npc1(-/-) mice is associated with increased Pcsk9 and Idol expression and decreased hepatic LDL-receptor levels. J Lipid Res 51:2655-63 (2010). WB ; Mouse . PubMed: 20562239
  • Capell BC  et al. A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model. Proc Natl Acad Sci U S A 105:15902-7 (2008). IHC-P ; Mouse . PubMed: 18838683
  • Song JH  et al. Subcellular targeting of RGS9-2 is controlled by multiple molecular determinants on its membrane anchor, R7BP. J Biol Chem 281:15361-9 (2006). PubMed: 16574655
  • Varga R  et al. Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome. Proc Natl Acad Sci U S A 103:3250-5 (2006). IHC-P ; Mouse . PubMed: 16492728

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1-5 of 5 Abreviews or Q&A

Western blot abreview for Anti-Lamin A antibody - Nuclear Envelope Marker

Average
Abreviews
Abreviews
abreview image
Application
Western blot
Sample
Mouse Tissue lysate - other (liver mitochondrial prep)
Loading amount
5 µg
Specification
liver mitochondrial prep
Gel Running Conditions
Reduced Denaturing
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Read More

Abcam user community

Verified customer

提交于 Jul 17 2007

Question

Could you please tell me if the following antibodies with cross with porcine lamin: ab2559; ab4789; ab5090; ab8980. Thanks

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Abcam community

Verified customer

Asked on Sep 27 2005

Answer

Thank you for your enquiry. All the information we have on species cross reactivity is specified on the datasheet, these are updated as soon as any new information is brought to our attention. As far as we are aware, cross reactivity with pig has not yet been tested for use with ab2559, ab4789, ab5090 or ab8980. Should you decide to go ahead and purchase one of these products, please let us know how you get on by submitting an Abreview and in return we will award you 50 Abcam Points, which can be redeemed on a number of rewards (a further 100 points will be offered for an image). Please let me know if you require any further assistance.

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Abcam Scientific Support

回复于 Sep 30 2005

Question

Customer's question: You say that your antibody ab2559 recognizes Lamin A (70kDa), but what is the cleaved band at 28kDa? I never heard about 28kDa nor 42kDa fragments. Could you explain me that?

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Abcam community

Verified customer

Asked on Mar 10 2005

Answer

Lamin A is cleaved by caspase-6 and thus as a marker for caspase-6 activation. During apoptosis , the 70 kDa lamin A can be cleaved to generate ~40-45 kDa large and 28 kDa small fragment. The cleavage of Lamins results in nuclear disregulation and cell death. If you search apoptosis and lamin A and caspase-6, you would find a lot references regarding the cleavage.

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Abcam Scientific Support

回复于 Mar 24 2005

Question

Thanks for your reply I did not personally run the western but I saw the result of human fibroblasts and there are a lot of bands on the western and it is difficult to see what is what. The same extract responds very well the chemicon anti lamin A+C antibody MAB3211 so the extract is of good quality. Question: since the ab2559 antibody is raised against a peptide corresponding to residues surrounding Asp230 and knowing that Asp230 is common to Lamin A & Lamin C, how do I know that ab2559 only recognizes Lamin A as it is specified on the product data sheet.

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Abcam community

Verified customer

Asked on Feb 11 2005

Answer

Thank you for your enquiry. The immunogen was from Lamin A (synthetic peptide -14 aa corresponding to residues surrounding Asp 230 of human Lamin A), but the antibody actually detects both Lamin A and Lamin C. The antibody detects uncleaved band ~70 kDa and cleaved band ~28 kDa.

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Abcam Scientific Support

回复于 Feb 16 2005

Question

Please can you tell me the exact immunogen used to raise this antibody? Does it recognise the 42 kDa cleavage product, also?

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Abcam community

Verified customer

Asked on Jun 30 2003

Answer

The immunogen used to raise this antibody is a synthetic peptide (14 aa) corresponding to residues surrounding Asp 230 of human Lamin A. It is therefore very unlikely that this antibody will detect the 42 kDa cleavage product. It will, however, detect the full length protein and small fragment (28 kDa) of Lamin A.

Read More

Abcam Scientific Support

回复于 Jul 01 2003

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

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