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Rabbit Anti-MYH7B/APC Conjugated antibody (bs-9862R-APC)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-9862R-APC
英文名稱 Rabbit Anti-MYH7B/APC Conjugated antibody
中文名稱 APC標(biāo)記的肌球蛋白重鏈7抗體
別    名 adult 1; Beta myosin heavy chain; cardiac muscle beta isoform; CMD1S; CMH1; MPD1; MYH1; MYH1_HUMAN; MYH7; MYH7_HUMAN; Myhc slow; MyHC-2x; MyHC-beta; MyHC-IIx/d; MyHC-slow; MYHCB; Myopathy, distal 1; Myosin heavy chain (AA 1-96); Myosin heavy chain 1; Myosin heavy chain 2x; Myosin heavy chain 7; Myosin heavy chain; Myosin heavy chain IIx/d; Myosin heavy chain slow isoform; Myosin heavy chain, cardiac muscle beta isoform; Myosin, heavy chain 7, cardiac muscle, beta; Myosin, heavy polypeptide 7, cardiac muscle, beta; Myosin-1; Myosin-7; Rhabdomyosarcoma antigen MU RMS 40.7A; skeletal muscle; SPMD.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 心血管  發(fā)育生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  干細(xì)胞  細(xì)胞骨架  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Horse, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MYH7
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
Myosin heavy chains are ubiquitous Actin-based motor proteins that convert the chemical energy derived from ATP hydrolysis into the mechanical energy that drives diverse motile processes in eukaryotic cells, including cytokinesis, vesicular transport and cellular locomotion. Muscle myosin is a heterohexamer consisting of two myosin heavy chains and two associated nonidentical pairs of myosin light chains. The seven myosin heavy chain isoforms that predominate in mammalian skeletal muscles include two developmental isoforms, MHC-embryonic (MYH3) and MHC-perinatal (MYH8); three adult skeletal muscle isoforms, MHC IIa (MYH2), MHC IIb (MYH4) and MHC IIx/d (MYH1); and MHC-∫/slow (MYH7 or MHC-∫), which is also expressed in cardiac muscle. Research indicates that mutations of the MYH7 gene causes hypertrophic cardiomyopathy.

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:
Defects in MYH7 are the cause of cardiomyopathy familial hypertrophic type 1 (CMH1) [MIM:192600]. 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 MYH7 are the cause of myopathy myosin storage (MYOMS) [MIM:608358]. In this disorder, muscle biopsy shows type 1 fiber predominance and increased interstitial fat and connective tissue. Inclusion bodies consisting of the beta cardiac myosin heavy chain are present in the majority of type 1 fibers, but not in type 2 fibers.


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

Database links:

Entrez Gene: 58498 Human

Omim: 612147 Human

SwissProt: Q01449 Human

Unigene: 75636 Human



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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