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肌球蛋白重鏈7抗體

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產品編號 Ys-9862R
英文名稱 MYH7
中文名稱 肌球蛋白重鏈7抗體
別    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.  

 

抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Chicken, Horse, )
產品應用 ELISA=1:5000-10000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (石蠟切片需做抗原修復)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 213kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MYH7: 1301-1500/1935 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
PubMed PubMed
產品介紹 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:
Myosins are actin-based motor molecules with ATPase activity essential for muscle contraction. Forms regular bipolar thick filaments that, together with actin thin filaments, constitute the fundamental contractile unit of skeletal and cardiac muscle.

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.
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