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Rabbit Anti-Myozenin 2/HRP Conjugated antibody (bs-18975R-HRP)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-18975R-HRP
英文名称 Anti-Myozenin 2/HRP
中文名称 辣根过氧化物酶标记的肌肉特异性蛋白MYOZ2抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,  )
产品应用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 30kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Myozenin 2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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