1. Signaling Pathways
  2. Membrane Transporter/Ion Channel
  3. CFTR

CFTR (囊性纤维化跨膜调节因子)

CFTR (Cystic fibrosis transmembrane conductance regulator), mutations of which cause cystic fibrosis, belongs to the ATP-binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. CFTR is composed of two homologous halves, each comprising a transmembrane (TMD) and a nucleotide binding domain (NBD). CFTR activity is regulated by phosphorylation of its cytosolic regulatory (R) domain, and ATP binding and hydrolysis at two NBDs.

CFTR is expressed in many cell types throughout the body, but in the airways it is found mainly in secretory serous cells of the submucosal glands. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. CFTR function is normally tightly controlled as dysregulation can lead to life-threatening diseases such as secretory diarrhoea and cystic fibrosis.

CFTR 相关产品 (2):

Cat. No. Product Name Effect Purity
  • HY-P1106A
    K41498 TFA Antagonist
    K41498 TFA 是一种强选择性的 CRF2 receptor 拮抗剂,其对人 CRF、CRF 和 CRF1 受体的 Ki 值分别为 0.66 nM、0.62 nM和425 nM。K41498 TFA 是 antisauvagine-30 (aSvg-30) 的类似物,可抑制 sauvagine 刺激的 cAMP 在 hCRF/hCRF 表达细胞中的积累。K41498 可用于低血压研究。
  • HY-103369
    PG01 Activator ≥98.0%
    PG01 是一种有效的 CFTR Cl- 通道增效剂。PG01 对 E193KG970RG551D (CFTR突变体) 有效,Kd 值分别为 0.22 μM,0.45 μM 和 1.94 μM。PG01对 ΔF508Ka 为 0.3 μM)也有效。PG01 在添加 Forskolin 后增加 ΔF508-CFTR Cl- 电流。
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