Recombinant Anti-Telethonin antibody [EPR8375] (ab133646)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8375] to Telethonin
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
Related conjugates and formulations
Overview
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Product name
Anti-Telethonin antibody [EPR8375]
See all Telethonin primary antibodies -
Description
Rabbit monoclonal [EPR8375] to Telethonin -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details
Unsuitable for: ICC/IF or IHC-P -
Species reactivity
Reacts with: Mouse, Rat, Human -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- Fetal muscle and fetal heart lysates
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General notes
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
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
Dissociation constant (KD)
KD = 1.30 x 10 -11 M Learn more about KD -
Storage buffer
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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Purity
Tissue culture supernatant -
Clonality
Monoclonal -
Clone number
EPR8375 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Isotype control
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab133646 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 |
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WB | (2) |
1/1000 - 1/10000. Predicted molecular weight: 19 kDa.
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Notes |
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WB
1/1000 - 1/10000. Predicted molecular weight: 19 kDa. |
Target
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Function
Muscle assembly regulating factor. Mediates the antiparallel assembly of titin (TTN) molecules at the sarcomeric Z-disk. -
Tissue specificity
Heart and skeletal muscle. -
Involvement in disease
Defects in TCAP are a cause of cardiomyopathy familial hypertrophic (CMH) [MIM:192600]; also designated FHC or HCM. 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 TCAP are a cause of limb-girdle muscular dystrophy type 2G (LGMD2G) [MIM:601954]. LGMD2G is an autosomal recessive degenerative myopathy characterized by proximal and distal muscle weakness and atrophy in the limbs, dystrophic changes on muscle biopsy, and absence of telethonin. Cardiac muscle is involved in a subset of patients.
Defects in TCAP are the cause of cardiomyopathy dilated type 1N (CMD1N) [MIM:607487]. 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. -
Cellular localization
Cytoplasm > myofibril > sarcomere. - Information by UniProt
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Database links
- Entrez Gene: 8557 Human
- Entrez Gene: 21393 Mouse
- Entrez Gene: 688173 Rat
- Omim: 604488 Human
- SwissProt: O15273 Human
- SwissProt: O70548 Mouse
- Unigene: 77628 Human
- Unigene: 10762 Mouse
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Alternative names
- 19 kDa sarcomeric protein antibody
- CMD1N antibody
- CMH25 antibody
see all
Images
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Lane 1 : Anti-Telethonin antibody [EPR8375] (ab133646) at 1/500 dilution
Lane 2 : Anti-Telethonin antibody [EPR8375] (ab133646) at 1/5000 dilution
Lane 1 : Rat Muscle tissue lysate
Lane 2 : Rat Heart tissue lysate
Lysates/proteins at 15 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 19 kDa
Observed band size: 19 kDa
Exposure time: 1 minuteBlocking buffer 5% NFDM/TBST
Diluting buffer 5% NFDM/TBST
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All lanes : Anti-Telethonin antibody [EPR8375] (ab133646) at 1/5000 dilution
Lane 1 : Human skeletal muscle tissue lysate
Lane 2 : C2C12 cell lysate
Lane 3 : Mouse heart tissue lysate
Lane 4 : Mouse muscle tissue lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 19 kDa
Observed band size: 19 kDa
Exposure time: 1 minuteBlocking buffer 5% NFDM/TBST
Diluting buffer 5% NFDM/TBST
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All lanes : Anti-Telethonin antibody [EPR8375] (ab133646) at 1/1000 dilution
Lane 1 : Fetal muscle lysate
Lane 2 : Fetal heart lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 19 kDa
Datasheets and documents
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SDS download
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Datasheet download
References (3)
ab133646 has been referenced in 3 publications.
- Lv X et al. Distal myopathy due to TCAP variants in four unrelated Chinese patients. Neurogenetics 22:1-10 (2021). PubMed: 32761539
- Denes LT et al. Microtubule-based transport is essential to distribute RNA and nascent protein in skeletal muscle. Nat Commun 12:6079 (2021). PubMed: 34707124
- Lawless M et al. Phosphodiesterase 5 inhibition improves contractile function and restores transverse tubule loss and catecholamine responsiveness in heart failure. Sci Rep 9:6801 (2019). PubMed: 31043634