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Tags & Cell Markers Subcellular Markers Nucleus Nuclear Envelope
使用敲除细胞株进行验证重组RabMAb

重组HRP Anti-Lamin A + Lamin C抗体[EPR4100] -核Envelope Marker (ab193904)

  • Datasheet
  • SDS
  • Certificate of Compliance
Submit a review Submit a question References (2)

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Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
  • Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
  • HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • HRP Rabbit monoclonal [EPR4100] to Lamin A + Lamin C - Nuclear Envelope Marker
  • Suitable for: IHC-P, WB
  • Knockout validated
  • Reacts with: Human
  • Conjugation: HRP

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概述

  • 产品名称

    HRP Anti-Lamin A + Lamin C抗体[EPR4100] -核Envelope Marker
    参阅全部 Lamin A + Lamin C 一抗
  • 描述

    HRP兔单克隆抗体[EPR4100] to Lamin A + Lamin C -核Envelope Marker
  • 宿主

    Rabbit
  • 偶联物

    HRP
  • 经测试应用

    适用于: IHC-P, WBmore details
  • 种属反应性

    与反应: Human
  • 免疫原

    Synthetic peptide within Human Lamin A + Lamin C aa 500-600. The exact sequence is proprietary.

  • 阳性对照

    • WB: HAP1, HeLa, HepG2, and SH-SY5Y whole cell lysate. IHC-P: FFPE human colon adenocarcinoma.
  • 常规说明

     

     

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

    Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

    Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

    We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

    In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

    We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

    Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

    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, as well as customer reviews and Q&As.

性能

  • 形式

    Liquid
  • 存放说明

    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. Store In the Dark.
  • 存储溶液

    pH: 7.40
    Preservative: 0.1% 10% Proclin 300 Solution
    Constituents: 30% Glycerol, 1% BSA, PBS
  • Concentration information loading...
  • 纯度

    Protein A purified
  • 克隆

    单克隆
  • 克隆编号

    EPR4100
  • 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

  • Alternative Versions

    • Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab108595)
    • Alexa Fluor® 488 Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab185014)
    • Alexa Fluor® 647 Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193903)
    • PE Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab210433)
    • Alexa Fluor® 594 Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab215324)
    • Anti-Lamin A + Lamin C antibody [EPR4100] - BSA and Azide free (ab216074)
  • Isotype control

    • HRP Rabbit IgG, monoclonal [EPR25A] - Isotype Control (ab199507)
  • Positive Controls

    • HeLa nuclear extract lysate (ab14655)

应用

Our Abpromise guarantee covers the use of ab193904 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-P 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
WB 1/5000. Detects a band of approximately 74 kDa (predicted molecular weight: 74 kDa).

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
    • Omim: 150330 Human
    • SwissProt: P02545 Human
    • Unigene: 594444 Human
    • 别名

      • 70 kDa lamin antibody
      • Cardiomyopathy dilated 1A (autosomal dominant) antibody
      • CDCD1 antibody
      • CDDC antibody
      • CMD1A antibody
      • CMT2B1 antibody
      • EMD2 antibody
      • FPL antibody
      • FPLD antibody
      • FPLD2 antibody
      • HGPS antibody
      • IDC antibody
      • Lamin A antibody
      • Lamin A/C antibody
      • Lamin A/C like 1 antibody
      • Lamin antibody
      • Lamin C antibody
      • lamin-a antibody
      • Lamin-A/C antibody
      • LDP1 antibody
      • LFP antibody
      • LGMD1B antibody
      • Limb girdle muscular dystrophy 1B (autosomal dominant) antibody
      • LMN 1 antibody
      • LMN A antibody
      • LMN C antibody
      • LMN1 antibody
      • LMNA antibody
      • LMNA_HUMAN antibody
      • LMNC antibody
      • LMNL1 antibody
      • Prelamin A/C antibody
      • PRO1 antibody
      • Renal carcinoma antigen NY REN 32 antibody
      • Renal carcinoma antigen NY-REN-32 antibody
      • Renal carcinoma antigen NYREN32 antibody
      see all

    图片

    • Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      All lanes : HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904) at 1/5000 dilution

      Lane 1 : Wild-type HAP1 cell lysate at 40 µg
      Lane 2 : LMNA knockout HAP1 cell lysate at 40 µg
      Lane 3 : HeLa cell lysate at 20 µg
      Lane 4 : HepG2 cell lysate at 20 µg

      Performed under reducing conditions.

      Predicted band size: 74 kDa
      Observed band size: 70-75 kDa
      why is the actual band size different from the predicted?


      Exposure time: 4 minutes


      ab193904 was shown to react with Anti-Lamin A + C antibody [EPR4100] - Nuclear Envelope Marker (HRP) in wild-type HAP1 cells in Western blot. Loss of signal was observed when LMNA knockout sample was used. Membranes were blocked in 3% milk in TBS-T (0.1% Tween®) before incubation with ab193904 overnight at 4°C at a 1 in 5000 dilution and ab184095 (Mouse Anti-GAPDH antibody [mAbcam 9484] - Alexa Fluor® 680) at a 1 in 1000 dilution. Blots were developed with Optiblot ECL reagent (ab133456) and imaged.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)

      IHC image of Lamin A + C staining in a section of formalin-fixed paraffin-embedded normal human colon*, performed on a Leica BOND™. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20mins. The section was then incubated with ab193904, 1/100 dilution, for 15 mins at room temperature. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX. The inset negative control image is taken from an identical assay without primary antibody.

      For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

      *Tissue obtained from the Human Research Tissue Bank, supported by the NIHR Cambridge Biomedical Research Centre

    • Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      Western blot - HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904) at 1/5000 dilution + SHSY-5Y (Human neuroblastoma cell line) Whole Cell Lysate at 10 µg

      Developed using the ECL technique.

      Performed under reducing conditions.

      Predicted band size: 74 kDa
      Observed band size: 74 kDa


      Exposure time: 20 minutes


      This blot was produced using a 4-12% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab193904 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.

    • HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)
      HRP Anti-Lamin A + Lamin C antibody [EPR4100] - Nuclear Envelope Marker (ab193904)

    实验方案

    • Western blot protocols
    • Immunohistochemistry protocols

    Click here to view the general protocols

    数据表及文件

    • Datasheet
    • SDS

    Certificate of Compliance

    To download a Certificate of Compliance, please enter your Lot number below:

  • 文献 (2)

    发表研究结果有使用 ab193904?请让我们知道,以便我们可以引用本数据表中的参考文章。

    ab193904 被引用在 2 文献中.

    • Schwertheim S  et al. New insights into intranuclear inclusions in thyroid carcinoma: Association with autophagy and with BRAFV600E mutation. PLoS One 14:e0226199 (2019). PubMed: 31841566
    • Zhang HT  et al. Andrographolide Induces Cell Cycle Arrest and Apoptosis of Chondrosarcoma by Targeting TCF-1/SOX9 Axis. J Cell Biochem N/A:N/A (2017). Mouse . PubMed: 28485543

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