Overview

  • Product name

  • Description

    Rabbit polyclonal to Titin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment corresponding to Human Titin aa 5398-5604.
    Sequence:

    VGSSICSARVTLREPPSFIKKIESTSSLRGGTAAFQATLKGSLPITVTWL KDSDEITEDDNIRMTFENNVASLYLSGIEVKHDGKYVCQAKNDAGIQRCS ALLSVKEPATITEEAVSIDVTQGDPATLQVKFSGTKEITAKWFKDGQELT LGSKYKISVTDTVSILKIISTEKKDSGEYTFEVQNDVGRSSCKARINVLD LIIPPSF


    Database link: Q8WZ42

  • Positive control

    • Mouse heart tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab193218 in the following tested applications.

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

Application Abreviews Notes
IHC-P 1/20 - 1/200.

Target

  • Function

    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.
  • Tissue specificity

    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.
  • Involvement in disease

    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.
  • Sequence similarities

    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.
  • Domain

    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.
  • Post-translational
    modifications

    Autophosphorylated (By similarity). Phosphorylated upon DNA damage, probably by ATM or ATR.
  • Cellular localization

    Cytoplasm. Nucleus.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Immunohistochemical analysis of paraffin-embedded mouse heart tissue labeling Titin with ab193218 at 1/50 dilution.

References

ab193218 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Immunocytochemistry/ Immunofluorescence
Sample
Dog Cell (Paraffin Heart)
Permeabilization
Yes - HIER - citrate buffer
Specification
Paraffin Heart
Blocking step
DAKO protein block as blocking agent for 10 minute(s) · Concentration: 100% · Temperature: RT°C
Fixative
10% NBF

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Verified customer

Submitted Nov 02 2016

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