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Troponin C (cTnC) (V3401-FITC)  
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100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 V3401-FITC
英文名稱 Troponin C (cTnC)
中文名稱 FITC標(biāo)記的心肌肌鈣蛋白單克隆抗體
別    名 troponin I type 3 (cardiac); Cardiac troponin I; Troponin I, cardiac muscle; Cardiomyopathy, familial hypertrophic, 7, included; CMD1FF; CMD2A; CMH7; cTnI; Familial hypertrophic cardiomyopathy 7; MGC116817; RCM1; Tn1; Tni; TNN I3; TNNC 1; TNNC-1; TNNC1; T  
克 隆 號 14F11K
理論分子量 23 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 >1mg/ml
緩 沖 液 0.01M PBS(pH7.4) with 0.1% Proclin300.
保存條件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 Troponin I is a cardiac and skeletal muscle protein useful in the laboratory diagnosis of heart attack. Troponin I is a part of the troponin protein complex, where it binds to actin in thin myofilaments to hold the actin-tropomyosin complex in place. Because of it, myosin cannot bind actin in relaxed muscle. When calcium binds to the troponin C it causes conformational changes which lead to dislocation of troponin I and finally tropomyosin leaves the binding site for myosin on actin leading to contraction of muscle.

Function:
Troponin I is the inhibitory subunit of troponin, the thin filament regulatory complex which confers calcium-sensitivity to striated muscle actomyosin ATPase activity.

Subunit:
Binds to actin and tropomyosin. Interacts with TRIM63. Interacts with STK4/MST1.

Post-translational modifications:
Phosphorylated at Ser-42 and Ser-44 by PRKCE; phosphorylation increases myocardium contractile dysfunction. Phosphorylated at Ser-23 and Ser-24 by PRKD1; phosphorylation reduces myofilament calcium sensitivity. Phosphorylated preferentially at Thr-31. Phosphorylation by STK4/MST1 alters its binding affinity to TNNC1 (cardiac Tn-C) and TNNT2 (cardiac Tn-T).

DISEASE:
Defects in TNNI3 are the cause of familial hypertrophic cardiomyopathy type 7 (CMH7) [MIM:613690]. 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 TNNI3 are the cause of familial restrictive cardiomyopathy type 1 (RCM1) [MIM:115210]. RCM1 is a heart muscle disorder characterized by impaired filling of the ventricles with reduced diastolic volume, in the presence of normal or near normal wall thickness and systolic function.
Defects in TNNI3 are the cause of cardiomyopathy dilated type 2A (CMD2A) [MIM:611880]. 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 TNNI3 are the cause of cardiomyopathy dilated type 1FF (CMD1FF) [MIM:613286]. 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.

Similarity:
Belongs to the troponin I family.

Database links:

Entrez Gene: 7137 Human

Omim: 191044 Human

SwissProt: P19429 Human

Unigene: 709179 Human



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