| 產(chǎn)品編號(hào) | bs-17086R |
| 英文名稱 | KBTBD13 Rabbit pAb |
| 中文名稱 | KBTBD13蛋白抗體 |
| 別 名 | 5430433E21Rik; HCG1645727; KBTBD_HUMAN; Kbtbd13; Kelch repeat and BTB(POZ) domain containing 13; Kelch repeat and BTB domain-containing protein 13; NEM6. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 發(fā)育生物學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Cow) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 49 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human KBTBD13: 381-458/458 |
| 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 |
The gene belongs to a family of genes encoding proteins containing a BTB domain and several kelch repeats. The BTB domain functions as a protein-protein interaction module, which includes an ability to self-associate or to interact with non-BTB domain-containing proteins. The kelch motif typically occurs in groups of five to seven repeats, and has been found in proteins with diverse functions. Known functions of these family members include transcription regulation, ion channel tetramerization and gating, protein ubiquitination or degradation, and cytoskeleton regulation. The exact function of this family member has yet to be determined. [provided by RefSeq, Jun 2010] Subcellular Location: Cytoplasm. Tissue Specificity: Expressed in skeletal muscle. DISEASE: Defects in KBTBD13 are the cause of nemaline myopathy type 6 (NEM6) [MIM:609273]. A form of nemaline myopathy characterized by childhood onset of slowly progressive proximal muscle weakness, exercise intolerance, and slow movements with stiff muscles. Patients are unable to run or correct themselves from falling over. Similarity: Contains 1 BTB (POZ) domain. Contains 5 Kelch repeats. SWISS: C9JR72 Gene ID: 390594 Database links: Entrez Gene: 390594 Human Omim: 613727 Human SwissProt: C9JR72 Human Unigene: 586890 Human |
