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Rabbit Anti-heavy chain cardiac Myosin/RBITC Conjugated antibody (bs-0259R-RBITC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-0259R-RBITC
英文名称 Anti-heavy chain cardiac Myosin/RBITC
中文名称 罗丹明(RBITC)标记的心肌肌球蛋白重链抗体
别    名 MYH6 + MYH7; MYH6 / MYH7; Alpha MHC; ASD3; CMD1S; CMH1; MGC138376; MGC138378; MPD1; MYH 6; MYH 7; MYH6; MYH7; MYHC A; MYHC; MYHC B; MyHC-alpha; MyHC-beta; MYHCA; MYHCB; Myosin heavy chain cardiac muscle alpha isoform; Myosin heavy chain cardiac muscle beta isoform; Myosin heavy polypeptide 7 cardiac muscle beta; MYH6_HUMAN; MYH7_HUMAN; heavy chain cardiac Myosin.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Chicken, Dog, Pig, Cow, Rabbit, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 213kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from the middle of human MyHC-beta
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:
Cardiac muscle myosin is a hexamer consisting of two heavy chain subunits, two light chain subunits, and two regulatory subunits. This gene encodes the alpha heavy chain subunit of cardiac myosin. The gene is located 4kb downstream of the gene encoding the beta heavy chain subunit of cardiac myosin. Mutations in this gene cause familial hypertrophic cardiomyopathy and atrial septal defect 3. [provided by RefSeq, Mar 2010].

Function:
Muscle contraction.

Subunit:
Muscle myosin is a hexameric protein that consists of 2 heavy chain subunits (MHC), 2 alkali light chain subunits (MLC) and 2 regulatory light chain subunits (MLC-2).

Subcellular Location:
Cytoplasm, myofibril. Note=Thick filaments of the myofibrils.

DISEASE:
Atrial septal defect 3 (ASD3) [MIM:614089]: A congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria. Note=The disease is caused by mutations affecting the gene represented in this entry.
Cardiomyopathy, familial hypertrophic 14 (CMH14) [MIM:613251]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
Cardiomyopathy, dilated 1EE (CMD1EE) [MIM:613252]: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Note=The disease is caused by mutations affecting the gene represented in this entry.
Sick sinus syndrome 3 (SSS3) [MIM:614090]: The term 'sick sinus syndrome' encompasses a variety of conditions caused by sinus node dysfunction. The most common clinical manifestations are syncope, presyncope, dizziness, and fatigue. Electrocardiogram typically shows sinus bradycardia, sinus arrest, and/or sinoatrial block. Episodes of atrial tachycardias coexisting with sinus bradycardia ('tachycardia-bradycardia syndrome') are also common in this disorder. SSS occurs most often in the elderly associated with underlying heart disease or previous cardiac surgery, but can also occur in the fetus, infant, or child without heart disease or other contributing factors. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry (PubMed:21378987). The lifetime risk of being diagnosed with sick sinus syndrome is higher for carriers of variant p.Arg721Trp than for non-carriers (PubMed:21378987).

Similarity:
Contains 1 IQ domain.
Contains 1 myosin head-like domain.

Database links:

Entrez Gene: 4624 Human

Entrez Gene: 4625 Human

Entrez Gene: 140781 Mouse

Entrez Gene: 17888 Mouse

Entrez Gene: 29556 Rat

Entrez Gene: 29557 Rat

Entrez Gene: 282714 Cow

Omim: 160710 Human

SwissProt: P12883 Human

SwissProt: P13533 Human

SwissProt: Q02566 Mouse

SwissProt: Q91Z83 Mouse

SwissProt: P02563 Rat

SwissProt: P02564 Rat

Unigene: 929 Human

Unigene: 319176 Mouse

Unigene: 127778 Rat



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

心肌肌球蛋白(cardiac myosin,CM)亦称肌凝蛋白,它是粗肌丝的主要成分,也是心肌细胞存在最多的结构蛋白。CM由一条重链(MHC)和两条轻链(MLC)两种亚单位组成,轻链分为轻链Ⅰ(MLC-Ⅰ)和轻链Ⅱ(MLC-Ⅱ)。CM在功能上与骨骼肌相似,但在氨基酸组织及结构上有差别。
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