製品の概要

  • 製品名Anti-Lamin A + C antibody [EPR4068]
    Lamin A + C 一次抗体 製品一覧
  • 製品の詳細
    Rabbit monoclonal [EPR4068] to Lamin A + C
  • 特異性The antibody recognizes full length Lamin A/C and the cleaved small unit.
  • アプリケーション適用あり: ICC/IF, Flow Cyt, WB, IP, IHC-Pmore details
  • 種交差性
    交差種: Mouse, Rat, Human, Spermophilus tridecemlineatus
  • 免疫原

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) corresponding to Human Lamin A + C aa 1-100.

  • ポジティブ・コントロール
    • WB: Mouse heart and rat heart tissue lysates and HeLa, HepG2 and HACAT cell lysates. IHC-P: Human ovarian carcinoma, human liver carcinoma, human breast, human uterus, human kidney mouse liver and rat liver tissues. ICC/IF: HeLa cells. Flow Cyt: HeLa cells. IP: HepG2 lysate.
  • 特記事項

    A trial size is available to purchase for this antibody.

    Produced using Abcam's RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5, 675, 063 and/or 7, 429, 487.

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    This product is a recombinant rabbit monoclonal antibody.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab108922 in the following tested applications.

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

アプリケーション Abreviews 特記事項
ICC/IF 1/100 - 1/250.
Flow Cyt 1/20.
WB 1/1000 - 1/10000. Predicted molecular weight: 74 kDa.
IP 1/10 - 1/100.
IHC-P 1/100 - 1/250.

ターゲット情報

  • 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
  • 参照データベース
  • 別名
    • 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/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

Anti-Lamin A + C antibody [EPR4068] 画像

  • All lanes : Anti-Lamin A + C antibody [EPR4068] (ab108922) at 1/10000 dilution (purified)

    Lane 1 : mouse heart lysate
    Lane 2 : rat heart lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    HRP goat anti-rabbit IgG (H+L) at 1/100000 dilution

    Predicted band size : 74 kDa
    Additional bands at : 65 kDa (possible isoform),70 kDa (possible isoform),74 kDa (possible isoform).

    Blocking buffer: 5% NFDM/TBST
    Dilution buffer: 5% NFDM/TBST

  • Immunofluorescence staining of HeLa cells with purified ab108922 at a working dilution of 1/250, counter-stained with DAPI. The secondary antibody was Alexa Fluor® 488 goat anti-rabbit (ab150077), used at a dilution of 1/1000. ab7291, a mouse anti-tubulin antibody (1/1000), was used to stain tubulin along with ab150120 (Alexa Fluor® 594 goat anti-mouse, 1/1000), shown in the top right hand panel. The cells were fixed in 4% PFA and permeabilized using 0.1% Triton X 100. The negative controls are shown in bottom middle and right hand panels - for negative control 1, purified ab108922 was used at a dilution of 1/500 followed by an Alexa Fluor® 594 goat anti-mouse antibody (ab150120) at a dilution of 1/500. For negative control 2, ab7291 (mouse anti-tubulin) was used at a dilution of 1/500 followed by an Alexa Fluor® 488 goat anti-rabbit antibody (ab150077) at a dilution of 1/400.

  • Immunohistochemical staining of paraffin embedded rat liver with purified ab108922 at a working dilution of 1/200. The secondary antibody used is HRP goat anti-rabbit IgG H&L (ab97051) at 1/500. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.
  • Flow Cytometry analysis of HeLa cells labelling Lamin A + C with purified ab108922 at a dilution of 1/20 (red). Cells were fixed with 4% paraformaldehyde and permeabilized with 90% methanol. An Alexa Flour® 488-conjugated goat anti-rabbit IgG (1/2000) was used as the secondary antibody. Black - Isotype control, rabbit monoclonal IgG. Blue - Unlabelled control, cells without incubation with primary and secondary antibodies.

  • ab108922 (purified) at 1/20 immunoprecipitating Lamin A + C in 10 μg HepG2 (Lanes 1 and 2, observed at 70, 74, and 65 kDa - ab108922 recognises three isoforms). Lane 3 - PBS. For western blotting, HRP Veriblot for IP (ab131366) was used as the secondary antibody (1/10 000). Blocking buffer and concentration: 5% NFDM/TBST Dilution buffer and concentration: 5% NFDM/TBST
  • Anti-Lamin A + C antibody [EPR4068] (ab108922) at 1/10000 dilution (purified) + HeLa cell lysate at 10 µg

    Secondary
    HRP goat anti-rabbit IgG (H+L) at 1/100000 dilution

    Predicted band size : 74 kDa
    Additional bands at : 65 kDa (possible isoform),70 kDa (possible isoform),74 kDa (possible isoform).

    Blocking buffer: 5% NFDM/TBST
    Dilution buffer: 5% NFDM/TBST

  • Immunohistochemical staining of paraffin embedded human ovarian carcinoma with purified ab108922 at a working dilution of 1/200. The secondary antibody used is HRP goat anti-rabbit IgG H&L (ab97051) at 1/500. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.
  • Immunohistochemical staining of paraffin embedded human liver carcinoma with purified ab108922 at a working dilution of 1/200. The secondary antibody used is HRP goat anti-rabbit IgG H&L (ab97051) at 1/500. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.
  • Immunohistochemical staining of paraffin embedded mouse liver with purified ab108922 at a working dilution of 1/200. The secondary antibody used is HRP goat anti-rabbit IgG H&L (ab97051) at 1/500. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.
  • Immunohistochemical analysis of paraffin-embedded human colonic tissue using unpurified ab108922 at a diltion of 1/100

  • All lanes : Anti-Lamin A + C antibody [EPR4068] (ab108922) at 1/1000 dilution (unpurified)

    Lane 1 : HeLa cell lysate
    Lane 2 : HepG2 cell lysate
    Lane 3 : HACAT cell lysate

    Lysates/proteins at 10 µg per lane.


    Predicted band size : 74 kDa
  • Unpurified ab108922 showing positive staining in Normal breast tissue.

  • Unpurified ab108922 showing positive staining in Normal uterus tissue.

  • Unpurified ab108922 showing positive staining in Normal kidney tissue.

Anti-Lamin A + C antibody [EPR4068] (ab108922) 使用論文

This product has been referenced in:
  • Yao Q  et al. C-terminal Src kinase (Csk)-mediated phosphorylation of eukaryotic elongation factor 2 (eEF2) promotes proteolytic cleavage and nuclear translocation of eEF2. J Biol Chem 289:12666-78 (2014). WB . Read more (PubMed: 24648518) »
  • Jung HJ  et al. Regulation of prelamin A but not lamin C by miR-9, a brain-specific microRNA. Proc Natl Acad Sci U S A 109:E423-31 (2012). IHC-Fr ; Mouse . Read more (PubMed: 22308344) »

See all 2 Publications for this product

Product Wall

Application Western blot
Sample Human Cell lysate - nuclear (HEK293 Lysate (kidney cell))
Gel Running Conditions Reduced Denaturing (4-12% Gradient)
Loading amount 5 µg
Specification HEK293 Lysate (kidney cell)
Blocking step Licor Blocking Reagent as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 4°C
Username

Dr. Sam Nowitzki

Verified customer

投稿 Jul 28 2015

Application Western blot
Loading amount 15 µg
Gel Running Conditions Reduced Denaturing
Sample Human Cell lysate - nuclear (HEK nuclear and cytoplasmic lysates)
Specification HEK nuclear and cytoplasmic lysates
Blocking step Licor blocking buffer as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 24°C
Username

Abcam user community

Verified customer

投稿 Jan 08 2015

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Spermophilus tridecemlineatus Cell lysate - nuclear (Brown adipose tissue)
Loading amount 30 µg
Specification Brown adipose tissue
Gel Running Conditions Reduced Denaturing (10% Tris-Glycine)
Blocking step BSA as blocking agent for 12 hour(s) and 0 minute(s) · Concentration: 5%
Username

Mr. James Bjork

Verified customer

投稿 Dec 19 2012

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"