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

CFTR

Cystic fibrosis transmembrane conductance regulator

CFTR

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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.

Cat. No. Product Name Effect Purity Chemical Structure
  • HY-14832S1
    Ataluren-13C6
    		Inhibitor
    Ataluren-13C6 (PTC124-13C6) is 13C labeled Ataluren. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.
    Ataluren-<sup>13</sup>C<sub>6</sub>
  • HY-P10226
    PGD97
    		Activator
    PGD97 is a selective cyclic peptide inhibitor against CAL/CFTR interactions, with a KD value of 6 nM towards the CAL PDZ domain for its desulfide cyclized form. PGD97 (desulfide cyclized form) has selectivity ≥ 130-fold compared to NHERF1/2 PDZ domains. PGD97 is capable of stabilizing F508del-CFTR at the cell membrane and improving CFTR function required for proper fluid homeostasis in tne lung. PGD97 can be used for the research of cystic fibrosis.
    PGD97
  • HY-B0679S
    Lubiprostone-d7
    		Activator
    Lubiprostone-d7 (RU-0211-d7) is the deuterium labeled Lubiprostone. Lubiprostone (SPI-0211) increases intestinal fluid secretion through generation of CIC-2/CFTR and activation of cAMP signaling pathway. Lubiprostone inhibits myeloperoxidase (MPO) activity, downregulates Indomethacin (HY-14397)-induced iNOS and TNFα expression. Lubiprostone can be used for chronic constipation research.
    Lubiprostone-d<sub>7</sub>
  • HY-108772
    Crofelemer
    		Modulator
    Crofelemer (Provir) is an orally active antidiarrheal agent. Crofelemer acts on the cystic fibrosis transmembrane conductance regulator (CFTR) and calcium-activated chloride channels (CACC) , which are responsible for chloride and fluid secretion in the gastrointestinal tract. Crofelemer can be used in the research of diarrheal diseases.
    Crofelemer
  • HY-14832R
    Ataluren (Standard)
    		Inhibitor
    Ataluren (Standard) is the analytical standard of Ataluren. This product is intended for research and analytical applications. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.
    Ataluren (Standard)
  • HY-15448S1
    Tezacaftor-d6
    		Modulator
    Tezacaftor-d6 (VX-661-d6) is deuterium labeled Tezacaftor. Tezacaftor (VX-661) is a F508del CFTR corrector. It helps CFTR protein reach the cell surface. However, Ivacaftor (VX-770, HY-13017), a CFTR potentiator, helps to prolong the opening time of cell surface CFTR protein channels. Tezacaftor combining with Ivacaftor, shows potent efficacy against cystic fibrosis and diseases with homozygous for the CFTR Phe508del mutation. Moreover, Elexacaftor (VX-445, HY-111772) is also a CFTR corrector. Elexacaftor-Tezacaftor-Ivacaftor aims at with cystic fibrosis (CF) with at least one Phe508del mutation, often avoids the indication for lung transplantation.
    Tezacaftor-d<sub>6</sub>
  • HY-B1125S
    Glucosamine-13C6
    Glucosamine-13C6 (D-Glucosamine-13C6) is 13C labeled Glucosamine. Glucosamine (D-Glucosamine) is an amino sugar and a prominent precursor in the biochemical synthesis of glycosylated proteins and lipids, is used as a dietary supplement. Glucosamine also is a natural constituent of glycosaminoglycans in the cartilage matrix and synovial fluid, which when administered exogenously, exerts pharmacological effects on osteoarthritic cartilage and chondrocytes.
    Glucosamine-<sup>13</sup>C<sub>6</sub>
Cat. No. Product Name / Synonyms Application Reactivity