抗体

异硫氰酸荧光素链接抗体肌球蛋白重链7, 心脏肌肉, 乙(MYH7)

MBS2016664

0.1毫克

260美元

多克隆

兔

异硫氰酸荧光素

免疫印迹, 酶联免疫吸附测定, 免疫细胞化学, 流式细胞仪, 免疫组化-石蜡切片, 免疫组化-冰冻切片

抗体

异硫氰酸荧光素链接抗体肌球蛋白重链7, 心脏肌肉, 乙(MYH7)

[肌球蛋白重链7, 心脏肌肉, 乙(MYH7)]

[myosin-7; Myosin-7; myosin-7; myHC-beta; myhc-slow; myopathy, distal 1; myosin heavy chain 7; beta-myosin heavy chain; myosin heavy chain (AA 1-96); myosin heavy chain slow isoform; rhabdomyosarcoma antigen MU-RMS-40.7A; myosin heavy chain, cardiac muscle beta isoform; myosin, heavy polypeptide 7, cardiac muscle, beta; myosin, heavy chain 7, cardiac muscle, beta; Myosin heavy chain 7; Myosin heavy chain slow isoform; MyHC-slow; Myosin heavy chain, cardiac muscle beta isoform]

[MYH7]

[MYH7; MYH7; CMH1; MPD1; SPMD; SPMM; CMD1S; MYHCB; MYHCB; MyHC-slow; MyHC-beta]

MYH7_HUMAN

多克隆

兔

人类

1935

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3, 50% glycerol.

200微克/毫升

Storage: Avoid repeated freeze/thaw cycles. Store at 4 degree C for frequent use. Aliquot and store at -20 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

免疫组化石蜡/冷冻(免疫组化-P/F), 免疫细胞化学(免疫细胞化学), 免疫荧光(IF), 免疫印迹(免疫印迹), 酶联免疫吸附测定

Western blotting: 0.5-2ug/ml. Immunocytochemistry in formalin fixed cells: 5-20ug/ml. Immunohistochemistry in formalin fixed frozen section: 5-20ug/ml. Immunohistochemistry in paraffin section: 5-20ug/ml. Enzyme-linked Immunosorbent Assay: 0.05-2ug/ml. Optimal working dilutions must be determined by end user

试验数据

Source: Antibody labeling. Label: FITC. Traits: Liquid. Immunogen: Recombinant Myosin Heavy Chain 7, Cardiac Muscle, Beta (MYH7)

Unconjugated Antibody: The unconjugated antibody version of this item is also available as catalog #MBS2007182

异硫氰酸荧光素抗体

115496169

NP_000248.2

NM_000257.2

P12883

223,097 Da

Adrenergic Signaling In Cardiomyocytes Pathway (908257); Adrenergic Signaling In Cardiomyocytes Pathway (909696); Cardiac Muscle Contraction Pathway (93344); Cardiac Muscle Contraction Pathway (93992); Dilated Cardiomyopathy Pathway (121494); Dilated Cardiomyopathy Pathway (121285); Hypertrophic Cardiomyopathy (HCM) Pathway (114229); Hypertrophic Cardiomyopathy (HCM) Pathway (106591); Membrane Trafficking Pathway (106160); Tight Junction Pathway (83071)

Muscle myosin is a hexameric protein containing 2 heavy chain subunits, 2 alkali light chain subunits, and 2 regulatory light chain subunits. This gene encodes the beta (or slow) heavy chain subunit of cardiac myosin. It is expressed predominantly in normal human ventricle. It is also expressed in skeletal muscle tissues rich in slow-twitch type I muscle fibers. Changes in the relative abundance of this protein and the alpha (or fast) heavy subunit of cardiac myosin correlate with the contractile velocity of cardiac muscle. Its expression is also altered during thyroid hormone depletion and hemodynamic overloading. Mutations in this gene are associated with familial hypertrophic cardiomyopathy, myosin storage myopathy, dilated cardiomyopathy, and Laing early-onset distal myopathy. [provided by RefSeq, Jul 2008]

MYH7: Muscle contraction. Defects in MYH7 are the cause of familial hypertrophic cardiomyopathy type 1 (CMH1). 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). 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. Defects in MYH7 are the cause of scapuloperoneal myopathy MYH7-related (SPMM); also known as scapuloperoneal syndrome myopathic type. SPMM is a progressive muscular atrophia beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. Defects in MYH7 are a cause of cardiomyopathy dilated type 1S (CMD1S). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Defects in MYH7 are the cause of myopathy distal type 1 (MPD1). MPD1 is 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. Protein type: Motor; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 14q12. Cellular Component: nucleoplasm; sarcomere; focal adhesion; cytoplasm; stress fiber; muscle myosin complex; Z disc; myosin complex. Molecular Function: calmodulin binding; microfilament motor activity; protein binding; ATPase activity; actin binding; actin-dependent ATPase activity; ATP binding. Biological Process: adult heart development; striated muscle contraction; muscle contraction; regulation of heart rate; metabolic process; ventricular cardiac muscle morphogenesis; regulation of the force of heart contraction; muscle filament sliding. Disease: Myopathy, Distal, 1; Scapuloperoneal Myopathy, Myh7-related; Myopathy, Myosin Storage; Cardiomyopathy, Dilated, 1s; Myopathy, Congenital, With Fiber-type Disproportion; Myopathy, Myosin Storage, Autosomal Recessive; Cardiomyopathy, Familial Hypertrophic, 1

0.1毫克

260美元

0.2毫克

400

1毫克

955