概述

  • 产品名称Anti-Titin抗体[T11]
    参阅全部 Titin 一抗
  • 描述
    小鼠单克隆抗体[T11] to Titin
  • 特异性Monoclonal Anti-Titin reacts only with skeletal and cardiac muscle, but not with smooth muscle or different non-muscle tissues and cultured cells. The antibody localizes titin (connectin) in skeletal and heart muscle of a wide variety of species from cold-blooded vertebrates to human. The product does not cross-react with nebulin.
  • 经测试应用适用于: WB, IHC-Fr, ICC/IF, Immunomicroscopy, Dot blot, Electron Microscopymore details
  • 种属反应性
    与反应: Rat, Chicken
    预测可用于: Rabbit, Human, Pig, Fish, a wide range of other species不与反应: Mouse, Cow
  • 免疫原

    Full length native protein (purified) (Chicken breast muscle).

  • 阳性对照
    • IHC-P: Rat soleus muscle tissue.
  • 常规说明

    Monoclonal Anti-Titin can be used for study of the elastic filaments within sarcomeric structures. It is also useful as a differentiation marker in the separation of rhabdomyosarcomas from other muscle tumors. By indirect immunofluorescence the antibody displays a typical striated staining pattern on frozen sections of chicken skeletal and cardiac muscle tissues. Stains the region of the A-I junction by indirect immunofluorescence. It shows a decoration line 0.05 mm from the end of the A band in electron micro-graphs. In immunoblotting, using total extracts of chicken breast muscle, the antibody reacts specifically with both bands of the titin double and shows no reaction with nebulin.

性能

应用

Our Abpromise guarantee covers the use of ab7034 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
WB Use at an assay dependent concentration. Predicted molecular weight: 2000 kDa.

Fish skeletal muscle +
Toad skeletal muscle +
Salamander skeletal muscle +
Salamander heart muscle +
Alligator skeletal muscle +
Pigeon skeletal muscle +
Chicken skeletal muscle +
Chicken heart muscle +
Chicken gizzard smooth muscle -
Mouse skeletal muscle -
Rat skeletal muscle +
Rat heart muscle +
Rat rhabdomyosarcoma +
Rabbit skeletal muscle +
Bovine heart muscle -
Pig skeletal muscle +
Human skeletal muscle +

IHC-Fr Use at an assay dependent concentration.
ICC/IF 1/1000.
Immunomicroscopy Use at an assay dependent concentration.
Dot blot Use at an assay dependent concentration.
Electron Microscopy Use at an assay dependent concentration.

靶标

  • 功能Key component in the assembly and functioning of vertebrate striated muscles. By providing connections at the level of individual microfilaments, it contributes to the fine balance of forces between the two halves of the sarcomere. The size and extensibility of the cross-links are the main determinants of sarcomere extensibility properties of muscle. In non-muscle cells, seems to play a role in chromosome condensation and chromosome segregation during mitosis. Might link the lamina network to chromatin or nuclear actin, or both during interphase.
  • 组织特异性Isoform 3, isoform 7 and isoform 8 are expressed in cardiac muscle. Isoform 4 is expressed in vertebrate skeletal muscle. Isoform 6 is expressed in cardiac tissues.
  • 疾病相关Defects in TTN are the cause of hereditary myopathy with early respiratory failure (HMERF) [MIM:603689]; also known as Edstrom myopathy. HMERF is an autosomal dominant, adult-onset myopathy with early respiratory muscle involvement.
    Defects in TTN are the cause of familial hypertrophic cardiomyopathy type 9 (CMH9) [MIM:613765]. Familial hypertrophic cardiomyopathy is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
    Defects in TTN are the cause of cardiomyopathy dilated type 1G (CMD1G) [MIM:604145]. 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 TTN are the cause of tardive tibial muscular dystrophy (TMD) [MIM:600334]; also known as Udd myopathy. TMD is an autosomal dominant, late-onset distal myopathy. Muscle weakness and atrophy are usually confined to the anterior compartment of the lower leg, in particular the tibialis anterior muscle. Clinical symptoms usually occur at age 35-45 years or much later.
    Defects in TTN are the cause of limb-girdle muscular dystrophy type 2J (LGMD2J) [MIM:608807]. LGMD2J is an autosomal recessive degenerative myopathy characterized by progressive weakness of the pelvic and shoulder girdle muscles. Severe disability is observed within 20 years of onset.
    Defects in TTN are the cause of early-onset myopathy with fatal cardiomyopathy (EOMFC) [MIM:611705]. Early-onset myopathies are inherited muscle disorders that manifest typically from birth or infancy with hypotonia, muscle weakness, and delayed motor development. EOMFC is a titinopathy that, in contrast with the previously described examples, involves both heart and skeletal muscle, has a congenital onset, and is purely recessive. This phenotype is due to homozygous out-of-frame TTN deletions, which lead to a total absence of titin's C-terminal end from striated muscles and to secondary CAPN3 depletion.
  • 序列相似性Belongs to the protein kinase superfamily. CAMK Ser/Thr protein kinase family.
    Contains 132 fibronectin type-III domains.
    Contains 152 Ig-like (immunoglobulin-like) domains.
    Contains 19 Kelch repeats.
    Contains 1 protein kinase domain.
    Contains 17 RCC1 repeats.
    Contains 14 TPR repeats.
    Contains 15 WD repeats.
  • 结构域ZIS1 and ZIS5 regions contain multiple SPXR consensus sites for ERK- and CDK-like protein kinases as well as multiple SP motifs. ZIS1 could adopt a closed conformation which would block the TCAP-binding site.
    The PEVK region may serve as an entropic spring of a chain of structural folds and may also be an interaction site to other myofilament proteins to form interfilament connectivity in the sarcomere.
  • 翻译后修饰Autophosphorylated (By similarity). Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 细胞定位Cytoplasm. Nucleus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • MPRM antibody
    • Cardiomyopathy dilated 1G (autosomal dominant) antibody
    • CMD1G antibody
    • CMH 9 antibody
    • CMH9 antibody
    • CMPD 4 antibody
    • CMPD4 antibody
    • Connectin antibody
    • DKFZp451N061 antibody
    • EOMFC antibody
    • FLJ26020 antibody
    • FLJ26409 antibody
    • FLJ32040 antibody
    • FLJ34413 antibody
    • FLJ39564 antibody
    • FLJ43066 antibody
    • HMERF antibody
    • LGMD2J antibody
    • MU RMS 40.14 antibody
    • MYLK5 antibody
    • Rhabdomyosarcoma antigen antibody
    • Rhabdomyosarcoma antigen MU RMS 40.14 antibody
    • Rhabdomyosarcoma antigen MU-RMS-40.14 antibody
    • Titin antibody
    • TITIN_HUMAN antibody
    • TMD antibody
    • TTN antibody
    see all

Anti-Titin antibody [T11] 图像

  • ab7034 at 1/100 staining rat soleus muscle tissue sections by IHC-Fr. The tissue was paraformaldehyde fixed and blocked with horse serum. A biotinylated horse anti-mouse IgG was used as the secondary.

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Anti-Titin antibody [T11] (ab7034)参考文献

This product has been referenced in:
  • Zhang Q  et al. Nesprin-2 is a multi-isomeric protein that binds lamin and emerin at the nuclear envelope and forms a subcellular network in skeletal muscle. J Cell Sci 118:673-87 (2005). IHC-Fr ; Human . Read more (PubMed: 15671068) »
  • Barta J  et al. Calpain-1-sensitive myofibrillar proteins of the human myocardium. Mol Cell Biochem 278:1-8 (2005). WB ; Human . Read more (PubMed: 16180082) »

See all 7 Publications for this product

Product Wall

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Dog Tissue sections (Heart)
Antigen retrieval step Heat mediated
Permeabilization No
Specification Heart
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 20°C
Fixative Formaldehyde
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Abcam user community

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提交于 Jul 28 2016

Abcam has not validated the combination of species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Human Tissue sections (human skeletal muscle sections)
Permeabilization Yes - Phosphate Buffer saline + 0.1% Trition X-100
Specification human skeletal muscle sections
Blocking step Serum as blocking agent for 30 minute(s) · Concentration: 10% · Temperature: 25°C
Fixative Methanol
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提交于 Apr 26 2016

Abcam has not validated the combination of species/application used in this Abreview.
Application Western blot
Sample Xenopus laevis Tissue lysate - whole (Xenopus laevis gastrocnemius muscle lysate)
Loading amount 60 µg
Specification Xenopus laevis gastrocnemius muscle lysate
Gel Running Conditions Reduced Denaturing (1% Agarose)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 23°C
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提交于 Apr 11 2013

Abcam has not validated the combination of species/application used in this Abreview.
Application Immunocytochemistry/ Immunofluorescence
Sample Xenopus laevis Cell (Nuclei generated in egg extract)
Specification Nuclei generated in egg extract
Fixative Formaldehyde
Permeabilization Yes - PBS-0.1% NP40
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 3% · Temperature: 23°C
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提交于 Apr 10 2013

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Rat Tissue sections (soleus muscle)
Specification soleus muscle
Fixative Paraformaldehyde
Blocking step Serum as blocking agent for 10 minute(s) · Concentration: 5 %
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提交于 Dec 13 2006

Thank you for your enquiry regarding separation on WB of large proteins. I would recommend using a 5% 1cm thick gel and to change the ratio of bis-acrylamide to mono-acrylamide from the classical 37:1 ratio to a 49:1 ratio. For the transfer I rec...

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Thank you for your enquiry. Development of the antigen for antibody production is a confidential/proprietary process which we do not have authority to detail at this time. However, you may find some useful information at the following websites:...

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ab7034 has not been used in this way and therefore we have no further information on whether it will be suitable for your application. We will update the on-line datasheet of this product as soon as we get more data. We apologize for any inconvenience ...

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Yes, you can use this antibody for Western blotting on human tissue samples. I am afraid that we don't know the size that Titin runs on a Western blot. I do know that the antibody detects a doublet though......we don't have a Western blot picture u...

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Yes, this antibody should cross react with human cardiac muscle. We have no information on the location of the epitope. The concentration of the antibody is 5.10 mg/ml - I'd start with a 1:2000 dilution. Price information in GBP/ USD/ EUR can be found ...

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