肌球蛋白7抗体
规格：1mg/1ml
英文名： Myosin-7
别名： MYH7_HUMAN; Myosin-7; Myosin heavy chain 7; Myosin heavy chain slow isoform; Short=MyHC-slow; Myosin heavy chain, cardiac muscle beta isoform; MyHC-beta.
分子量： 223kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Myosin-7
交叉反应：Human,
细胞定位：
肌球蛋白7抗体产品介绍：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). Interacts with ECM29. Subcellular Location: Cytoplasm, myofibril. Note=Thick filaments of the myofibrils. Tissue Specificity: Both wild type and variant Gln-403 are detected in skeletal muscle (at protein level). DISEASE: Cardiomyopathy, familial hypertrophic 1 (CMH1) [MIM:192600]: 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 肌球蛋白7抗体in this entry. Myopathy, myosin storage (MYOMS) [MIM:608358]: A rare congenital myopathy characterized by subsarcolemmal hyalinized bodies in type 1 muscle fibers. Note=The disease is caused by mutations affecting the gene represented in this entry. Scapuloperoneal myopathy MYH7-related (SPMM) [MIM:181430]: Progressive muscular atrophia beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. Note=The disease is caused by mutations affecting the gene represented in this entry. Cardiomyopathy, dilated 1S (CMD1S) [MIM:613426]: 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. Myopathy肌球蛋白7抗体, distal, 1 (MPD1) [MIM:160500]: A muscular disorder characterized by early-onset selective weakness of the great toe and ankle dorsiflexors, followed by weakness of the finger extensors. Mild proximal weakness occasionally develops years later after the onset of the disease. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Contains 1 IQ domain. Contains 1 myosin head-like domain. Gene ID: 4625 Database links: Entrez Gene: 4625 Human Entrez Gene: 140781 Mouse Entrez Gene: 29557 Rat Omim: 160760 Human SwissProt: P12883 Human SwissProt: Q91Z83 Mouse SwissProt: P02564 Rat Unigene: 719946 Human Unigene: 457983 Mouse Unigene: 225886 Rat Unigene: 54399 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
肌球蛋白7抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid