General description
The induced pluripotent stem cell (iPSC) line derived from cystic fibrosis (CF) patients with the G542X mutation represents a significant advancement in CF research. This line features the G542X mutation, a nonsense mutation in the CFTR gene that leads to a premature stop codon, resulting in the production of a truncated, nonfunctional cystic fibrosis transmembrane conductance regulator (CFTR) protein. This disruption hampers chloride ion transport, leading to thick mucus accumulation in the lungs and pancreas, which is characteristic of cystic fibrosis.The G542X iPSC line provides critical advantages for cystic fibrosis research. It enables direct studies of the molecular mechanisms underlying CF and elucidates the specific cellular dysfunctions caused by the mutation. Importantly, these iPSCs can be differentiated into organoids that mimic the structure and function of human tissues affected by cystic fibrosis, such as lung and intestinal organoids.These organoids serve as valuable models for studying the pathophysiology of cystic fibrosis, allowing researchers to investigate how the G542X mutation impacts cellular behavior and tissue function. Additionally, organoids can be used to screen potential therapeutic compounds, enabling the evaluation of drug efficacy in a more physiologically relevant context. This approach facilitates the development of targeted treatments aimed at restoring CFTR function and improving patient outcomes.Overall, the availability of the G542X iPSC line and the organoids derived from it empowers researchers to advance cystic fibrosis research and promote innovative treatment strategies that address the genetic basis of the disease.
Application
Each vial contains >1X106 viable cells.
Cells are tested negative for HPV-16, HPV-18, Hepatitis A, B, C, and HIV-1 & 2 viruses by PCR.
Cells are negative for mycoplasma contamination.
Each lot of cells is genotyped by STR analysis to verify the unique identity of the cell line.
Features and Benefits
Human induced pluripotent stem cell (iPSC) line derived from a cystic fibrosis (CF) patient with G542X mutation.
Preparation Note
Human iPS cells should be stored in liquid nitrogen. The cells can be cultured for at least 10 passages after initial thawing without significantly affecting the cell marker expression and functionality.
Other Notes
This product is intended for sale and sold solely to academic institutions for internal academic research use per the terms of the “Academic Use Agreement” as detailed in the product documentation. For information regarding any other use, please contact
[email protected].
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
