製品の概要

  • 製品名Anti-Lamin A + C antibody [JOL2]
    Lamin A + C 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal [JOL2] to Lamin A + C
  • 特異性This antibody reacts with both recombinant and native Lamin A and C in humans.
  • アプリケーション適用あり: ICC/IF, IHC-Fr, IHC-P, WB, IP, Flow Cytmore details
  • 種交差性
    交差種: Human, African Green Monkey
  • 免疫原

    Recombinant fragment corresponding to Human Lamin A + C.

  • エピトープThis product has been shown to bind to an epitope between amino acids 464-572.
  • ポジティブ・コントロール
    • Human young placenta, tonsil.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab40567 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 Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
IHC-P Use at an assay dependent concentration.
WB 1/200. Predicted molecular weight: 74 kDa.
IP Use at an assay dependent concentration.
Flow Cyt 1/10. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.

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 [JOL2] 画像

  • Anti-Lamin A + C antibody [JOL2] (ab40567) at 1/200 dilution + HeLa whole cell lysate (10ug)

    Secondary
    HRP conjugated goat anti-mouse antibody
    Developed using the ECL technique

    Performed under non-reducing conditions.

    Predicted band size : 74 kDa
    Observed band size : 64,73 kDa (why is the actual band size different from the predicted?)


    Exposure time : 10 seconds

    This image is courtesy of an anonymous Abreview

    See Abreview

  • Overlay histogram showing HeLa cells stained with ab40567 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab40567, 1/10 dilution) for 30 min at 22°C. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22°C. Isotype control antibody (black line) was mouse IgG1 [ICIGG1](ab91353, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed.

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human colon tissue sections labelling Lamin A + C with ab40567.

  • ab40567 staining Lamin A + C in HeLa cells by Immunocytochemistry/ Immunofluorescence.
    Cells were fixed in 100% methanol, blocked with 5% serum for 30 minutes at 22°C and then incubated with ab40567 at a 1/100 dilution for 1 hour 30 minutes at 22°C. The secondary used was ab98795, donkey anti-mouse IgG - H&L (DyLight® 550), pre-adsorbed, used at a 1/200 dilution. DAPI was used to counterstain nuclei.

    See Abreview

  • ab40567 staining Lamin A + C in African Green Monkey CV1 cells by Immunocytochemistry/ Immunofluorescence.
    Cells were fixed in 100% methanol, blocked with 5% serum for 30 minutes at 22°C and then incubated with ab40567 at a 1/100 dilution for 1 hour 30 minutes at 22°C. The secondary used was ab98795, donkey anti-mouse IgG - H&L (DyLight® 550), pre-adsorbed, used at a 1/200 dilution. DAPI was used to counterstain nuclei.

    See Abreview

Anti-Lamin A + C antibody [JOL2] (ab40567) 使用論文

This product has been referenced in:
  • Zhang Y  et al. Human skeletal muscle xenograft as a new preclinical model for muscle disorders. Hum Mol Genet N/A:N/A (2014). Human . Read more (PubMed: 24452336) »
  • Ferreboeuf M  et al. Nuclear protein spreading: implication for pathophysiology of neuromuscular diseases. Hum Mol Genet N/A:N/A (2014). Read more (PubMed: 24659496) »

See all 12 Publications for this product

Product Wall

Application Western blot
Loading amount 20 µg
Gel Running Conditions Non-reduced Non-Denaturing (Native) (precast 4-15%)
Sample Human Cell lysate - whole cell (breast cell line)
Specification breast cell line
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Abcam user community

Verified customer

投稿 Mar 11 2014

Here is a link to a reference that used the antibody for IP. I hope this helps. Please let us know if you have any questions.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC138642/

Mouse IgG Dynabeads (Dynal Biotech, Oslo, Norway) we...

Read More

You are correct to wonder about the differences between these two products. They are the same antibody clone, JOL2, and the products differ mainly in the formulation: ab40567 has sodium azide as a preservative, and ab106708 has no preservative, which i...

Read More
Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunocytochemistry/ Immunofluorescence
Sample African Green Monkey Cell (CV1)
Specification CV1
Fixative Methanol
Permeabilization Yes - Methanol
Blocking step Serum as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 22°C
Username

Mr. Nir Drayman

Verified customer

投稿 Nov 21 2011

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunocytochemistry/ Immunofluorescence
Sample Human Cell (HeLa)
Specification HeLa
Fixative Methanol
Permeabilization Yes - Methanol
Blocking step Serum as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 22°C
Username

Mr. Nir Drayman

Verified customer

投稿 Nov 18 2011

Application Western blot
Sample Human Cell lysate - nuclear (SHSY5Y cell)
Loading amount 20 µg
Specification SHSY5Y cell
Gel Running Conditions Reduced Denaturing
Blocking step Milk as blocking agent for 3 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Abcam user community

Verified customer

投稿 May 09 2011

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (HeLa)
Loading amount 10 µg
Specification HeLa
Blocking step Milk as blocking agent for 2 hour(s) and 0 minute(s) · Concentration: 5% milk/TB
Username

Abcam user community

Verified customer

投稿 Mar 20 2007

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