| 產(chǎn)品編號 | bsm-42234R |
| 英文名稱 | Myosin VIIa Recombinant Rabbit mAb |
| 中文名稱 | 肌球蛋白7a/常染色體隱性耳聾蛋白2重組兔單抗 |
| 別 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. |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號 | 19C1 |
| 交叉反應(yīng) | Mouse |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 244 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | Recombinant mouse Myosin VIIa protein: 861-1035/2215 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. Database links: UniProtKB/Swiss-Prot: Q13402.2 |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with Myosin VIIa monoclonal antibody, unconjugated (bsm-42234R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Sample:
Lane 1: Recombinant mouse Myosin VIIa protein, N-Trx-His(bs-42234P)
Primary: Anti-Myosin VIIa (bsm-42234R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 244 kDa
Observed band size: 42 kDa
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