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Myozenin 2 Rabbit pAb (bs-18975R)  
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產(chǎn)品編號(hào) bs-18975R
英文名稱 Myozenin 2 Rabbit pAb
中文名稱 肌肉特異性蛋白MYOZ2抗體
別    名 C4orf5; Calcineurin binding protein calsarcin 1; Calsarcin 1; Calsarcin-1; Calsarcin1; CMH16; CS 1; CS1; FATZ related protein 2; FATZ-related protein 2; Muscle specific protein; MYOZ 2; MYOZ2; MYOZ2_HUMAN; Myozenin-2; Myozenin2.  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,Rat (predicted: Human,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 30 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Myozenin 2: 1-100/264 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The protein encoded by this gene belongs to a family of sarcomeric proteins that bind to calcineurin, a phosphatase involved in calcium-dependent signal transduction in diverse cell types. These family members tether calcineurin to alpha-actinin at the z-line of the sarcomere of cardiac and skeletal muscle cells, and thus they are important for calcineurin signaling. Mutations in this gene cause cardiomyopathy familial hypertrophic type 16, a hereditary heart disorder. [provided by RefSeq, Aug 2011]

Function:
Myozenins may serve as intracellular binding proteins involved in linking Z line proteins such as alpha-actinin, gamma-filamin, TCAP/telethonin, LDB3/ZASP and localizing calcineurin signaling to the sarcomere. Plays an important role in the modulation of calcineurin signaling. May play a role in myofibrillogenesis.

Subcellular Location:
Cytoplasm > myofibril > sarcomere > Z line. Colocalizes with ACTN1 and PPP3CA at the Z-line of heart and skeletal muscle.

Tissue Specificity:
Expressed specifically in heart and skeletal muscle.

DISEASE:
Defects in MYOZ2 are the cause of familial hypertrophic cardiomyopathy type 16 (CMH16) [MIM:613838]. CMH16 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.

Similarity:
Belongs to the myozenin family.

SWISS:
Q9NPC6

Gene ID:
51778

Database links:

Entrez Gene: 51778 Human

Entrez Gene: 540487 Cow

Entrez Gene: 59006 Mouse

Entrez Gene: 295426 Rat

Entrez Gene: 100526817 Sheep

Omim: 605602 Human

SwissProt: Q5E9V3 Cow

SwissProt: Q9NPC6 Human

SwissProt: Q9JJW5 Mouse

Unigene: 732122 Human

Unigene: 141157 Mouse

Unigene: 12931 Rat



產(chǎn)品圖片
Sample: Lane 1: Mouse Heart tissue lysates Lane 2: Mouse Cerebrum tissue lysates Lane 3: Rat Cerebrum tissue lysates Primary: Anti-Myozenin 2 (bs-18975R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size
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