| 产品编号 | bs-8257R |
| 英文名称 | TBX1 Rabbit pAb |
| 中文名称 | 先心病相关蛋白TBX1抗体 |
| 别 名 | CAFS; CTHM; DGCR; DGS; DORV; T box 1; T box 1 transcription factor; T box 1 transcription factor C; T box; T box protein 1; T box transcription factor TBX 1; T box transcription factor TBX1; T-box 1; T-box protein 1; T-box transcription factor TBX1; TBX 1; TBX 1C; tbx1; TBX1_HUMAN; TBX1C; Testis specific T box protein; Testis-specific T-box protein; TGA; VCFS. |
| 研究领域 | 心血管 细胞生物 免疫学 神经生物学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 克 隆 号 | |
| 交叉反应 | Human,Mouse,Rat (predicted: Pig,Cow,Chicken,Dog,Horse) |
| 产品应用 | WB=1:500-2000,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 | 43 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞核 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human TBX1: 165-270/398 |
| 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 |
This gene is a member of a phylogenetically conserved family of genes that share a common DNA-binding domain, the T-box. T-box genes encode transcription factors involved in the regulation of developmental processes. This gene product shares 98% amino acid sequence identity with the mouse ortholog. DiGeorge syndrome (DGS)/velocardiofacial syndrome (VCFS), a common congenital disorder characterized by neural-crest-related developmental defects, has been associated with deletions of chromosome 22q11.2, where this gene has been mapped. Studies using mouse models of DiGeorge syndrome suggest a major role for this gene in the molecular etiology of DGS/VCFS. Several alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2008]. Function: Probable transcriptional regulator involved in developmental processes. Is required for normal development of the pharyngeal arch arteries. Subunit: Interacts with DSCR6. Subcellular Location: Nucleus. DISEASE: Note=Haploinsufficiency of the TBX1 gene is responsible for most of the physical malformations present in DiGeorge syndrome (DGS) and velocardiofacial syndrome (VCFS). DGS is characterized by the association of several malformations: hypoplastic thymus and parathyroid glands, congenital conotruncal cardiopathy, and a subtle but characteristic facial dysmorphology. VCFS is marked by the association of congenital conotruncal heart defects, cleft palate or velar insufficiency, facial dysmorpholgy and learning difficulties. It is now accepted that these two syndromes represent two forms of clinical expression of the same entity manifesting at different stages of life. DiGeorge syndrome (DGS) [MIM:188400]: A congenital syndrome characterized by a wide spectrum of characteristics including parathyroid hypoplasia resulting in hypocalcemia, thymic hypoplasia resulting in T-cell immunodeficiency, defects in the outflow tract of the heart, and craniofacial anomalies. Disturbance of cervical neural crest migration into the derivatives of the pharyngeal arches and pouches can account for the phenotype. Note=The disease is caused by mutations affecting the gene represented in this entry. Velocardiofacial syndrome (VCFS) [MIM:192430]: A syndrome characterized by abnormal pharyngeal arch development that results in defective development of the parathyroid glands, thymus, and conotruncal region of the heart. The phenotype is highly variable, with no single clinical feature present in every patient. Affected individuals may present with structural or functional palatal abnormalities, cardiac defects, unique facial characteristics, hypernasal speech, hypotonia, and defective thymic development associated with impaired immune function. In addition, affected individuals may present with learning disabilities, overt developmental delay, and psychiatric disorders. Note=The disease is caused by mutations affecting the gene represented in this entry. Conotruncal heart malformations (CTHM) [MIM:217095]: A group of congenital heart defects involving the outflow tracts. Examples include truncus arteriosus communis, double-outlet right ventricle and transposition of great arteries. Truncus arteriosus communis is characterized by a single outflow tract instead of a separate aorta and pulmonary artery. In transposition of the great arteries, the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. In double outlet of the right ventricle, both the pulmonary artery and aorta arise from the right ventricle. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Contains 1 T-box DNA-binding domain. SWISS: O43435 Gene ID: 6899 Database links: Entrez Gene: 6899 Human Entrez Gene: 21380 Mouse Omim: 602054 Human SwissProt: O43435 Human SwissProt: P70323 Mouse Unigene: 173984 Human Unigene: 295194 Mouse Unigene: 41347 Rat |
| 产品图片 |
Sample: Muscle (Mouse) Lysate at 40 ug
Primary: Anti-TBX1 (bs-8257R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 43 kD
Observed band size: 43 kD
Sample: Brain (Rat) Lysate at 40 ug
Primary: Anti-TBX1 (bs-8257R) at 1/300 dilution
Secondary: HRP conjugated Goat-Anti-rabbit IgG (bs-0295G-HRP) at 1/5000 dilution
Predicted band size: 43 kD
Observed band size: 44 kD
Sample:
Lane 1: Mouse Heart tissue lysates
Lane 2: Rat Heart tissue lysates
Lane 3: Human K562 cell lysates
Lane 4: Human 293T cell lysates
Primary: Anti-TBX1 (bs-8257R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 43 kDa
Observed band size: 43 kDa
Blank control:K562.
Primary Antibody (green line): Rabbit Anti-TBX1 antibody (bs-8257R)
Dilution: 2μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody : Goat anti-rabbit IgG-PE
Dilution: 1μg /test.
Protocol
The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at-20℃. 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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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
