验证数据展示
产品信息
20738-1-PBS targets CFTR in IHC, FC (Intra), Indirect ELISA applications and shows reactivity with human samples.
| 经测试应用 | IHC, FC (Intra), Indirect ELISA Application Description |
| 经测试反应性 | human |
| 免疫原 |
Peptide 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7) |
| 别名 | ABCC7, ATP-binding cassette sub-family C member 7, cAMP-dependent chloride channel, CFTR/MRP, Channel conductance-controlling ATPase |
| 计算分子量 | 168 kDa |
| GenBank蛋白编号 | NM_000492 |
| 基因名称 | CFTR |
| Gene ID (NCBI) | 1080 |
| RRID | AB_2722558 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | P13569 |
| 储存缓冲液 | PBS only, pH 7.3. |
| 储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
What is the human cytogenetic location of the CFTR gene?
7q31.2
What is the predicted molecular weight of CFTR?
168 kDa
Where is CFTR expressed?
CFTR is mainly expressed in apical membranes of vertebrate epithelial cells.
What is the ion selectivity of CFTR?
CFTR is an anion-selective channel, responsible for regulating chloride and bicarbonate transport.
What is the process of CFTR trafficking?
The ER provides the first steps in CFTR folding quality control. CFTR can be degraded by the ER associated degradation (ERAD) pathway, or it is transported to the Golgi for maturation before being trafficked to the cell membrane.
What are the separate domains of CFTR?
CFTR consists of two transmembrane domains, two nucleotide binding domains, and a regulatory domain.
What regulates CFTR?
CFTR activity is ATP-dependent and is regulated by protein kinase A phosphorylation.
What is the role of CFTR?
CFTR functions as a channel for anions, regulating water and ionic homeostasis.
What mutations in CFTR leads to protein degradation?
Deletion of the regulatory domain or the phenylalanine 508 (F508del) results in an increase in CFTR degradation. This degradation then leads to a decrease of secretion product clearance and an increase in viscous mucosal buildup at the epithelial surface of affected organs, the primary symptom of cystic fibrosis. Mutations in CFTR can also lead congenital bilateral absence of vas deferens (CBAVD) or sperm abnormalities in men.
How does CFTR differ from other ATP-binding cassette proteins?
Unlike other proteins in the ABC family, CFTR functions as an ion channel.
PMIDs: 24513531, 24534272, 22966013, 22709980
