重组Anti-Telethonin抗体[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
概述
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产品名称
Anti-Telethonin抗体[EPR8375]
参阅全部 Telethonin 一抗 -
描述
兔单克隆抗体[EPR8375] to Telethonin -
宿主
Rabbit -
经测试应用
适用于: WBmore details
不适用于: ICC/IF or IHC-P -
种属反应性
与反应: Mouse, Rat, Human -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- Fetal muscle and fetal heart lysates
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常规说明
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.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
解离常数(KD)
KD = 1.30 x 10 -11 M Learn more about KD -
存储溶液
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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纯度
Tissue culture supernatant -
克隆
单克隆 -
克隆编号
EPR8375 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab133646于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB | (2) |
1/1000 - 1/10000. Predicted molecular weight: 19 kDa.
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说明 |
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WB
1/1000 - 1/10000. Predicted molecular weight: 19 kDa. |
靶标
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功能
Muscle assembly regulating factor. Mediates the antiparallel assembly of titin (TTN) molecules at the sarcomeric Z-disk. -
组织特异性
Heart and skeletal muscle. -
疾病相关
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. -
细胞定位
Cytoplasm > myofibril > sarcomere. - Information by UniProt
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数据库链接
- 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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别名
- 19 kDa sarcomeric protein antibody
- CMD1N antibody
- CMH25 antibody
see all
图片
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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
数据表及文件
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SDS download
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Datasheet download
文献 (3)
ab133646 被引用在 3 文献中.
- 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