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Myosin VIIa Rabbit pAb (bs-7761R)  
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产品编号 bs-7761R
英文名称 Myosin VIIa Rabbit pAb
中文名称 肌球蛋白7a/常染色体隐性耳聋蛋白2抗体
别    名 Deafness autosomal dominant 11; Deafness autosomal recessive 2; DFNA11; DFNB 2; DFNB2; Myo7a; Myosin 7a; Myosin VIIa; MYU7A; NSRD 2; NSRD2; Ush 1B; Ush1b; Usher syndrome 1B.  
研究领域 信号转导  干细胞  细胞骨架  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,Mouse (predicted: Rat,Pig,Cow,Chicken,Dog,Horse)
产品应用 Flow-Cyt=2ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 244 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Myosin VIIa: 851-950/2215 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 Myosins are actin-based motor molecules with ATPase activity. Unconventional myosins serve in intracellular movements. Their highly divergent tails are presumed to bind to membranous compartments, which would be moved relative to actin filaments. In retina, myosin VIIa may play a role in trafficking of ribbon-synaptic vesicle complexes and renewal of the outer photoreceptors disks. In inner ear, it may maintain the rigidity of stereocilia during the dynamic movements of the bundle.

Function:
Myosins are actin-based motor molecules with ATPase activity. Unconventional myosins serve in intracellular movements. Their highly divergent tails are presumed to bind to membranous compartments, which would be moved relative to actin filaments. In retina, myosin VIIa might play a role in trafficking of ribbon-synaptic vesicle complexes and renewal of the outer photoreceptors disks. In inner ear, it might maintain the rigidity of stereocilia during the dynamic movements of the bundle. Involved in hair-cell vesicle trafficking of aminoglycosides, which are known to induce ototoxicity.

Subunit:
Interacts with PLEKHB1 (via PH domain). Might homodimerize in a two headed molecule through the formation of a coiled-coil rod. Binds MYRIP and WHRN.

Subcellular Location:
Cytoplasm (Probable). Note=In the photoreceptor cells, mainly localized in the inner and base of outer segments as well as in the synaptic ending region.

Tissue Specificity:
Expressed in the pigment epithelium and the photoreceptor cells of the retina. Also found in kidney, liver, testis, cochlea, lymphocytes. Not expressed in brain.

DISEASE:
Defects in MYO7A are the cause of Usher syndrome type 1B (USH1B) [MIM:276900]. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa and sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness.
Defects in MYO7A are the cause of deafness autosomal recessive type 2 (DFNB2) [MIM:600060]; also called neurosensory non-syndromic recessive deafness 2 (NSRD2). DFNB2 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Defects in MYO7A are the cause of deafness autosomal dominant type 11 (DFNA11) [MIM:601317].

Similarity:
Contains 2 FERM domains.
Contains 5 IQ domains.
Contains 1 myosin head-like domain.
Contains 2 MyTH4 domains.
Contains 1 SH3 domain.

SWISS:
Q13402

Gene ID:
4647

Database links:
 

UniProtKB/Swiss-Prot: Q13402.2



产品图片
Blank control:Mouse kidney. Primary Antibody (green line): Rabbit Anti-Myosin VIIa antibody (bs-7761R) Dilution: 2μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-AF488 Dilution: 1μg /test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 0.1% PBST for 20 min at room temperature. The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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