Cystic Fibrosis (CF) is a progressive genetic disease that affects the lungs and digestive system as...

Cystic Fibrosis (CF) is a progressive genetic disease that affects the lungs and digestive system as well as many other organ systems. It causes thick, sticky mucus to build up in the lungs, which leads to life-threatening lung infections. Cystic fibrosis is caused by mutations in the gene that produces the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This protein is responsible for regulating the flow of salt and fluids in and out of the cells in different parts of the body. Induced pluripotent Stem (iPS) cells might offer hope for curing CF. Describe how iPS cell technology can be used to treat individuals suffering from CF. Note: Your answer should indicate the most appropriate cells taken from the patient for conversion to iPS cells and the genes that need to be expressed or repaired.

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Induced pluripotent stem cells are a type of cells that can self renew by dividing and developinto th eprimary germ cells of the early embryo. It can be generated directly from a somatic cell. They can give rise to any type of cell in our body like the heart, liver, neurons or pancreas. They represent a single source of cells that can be used to replace anothe organ or cells that have bbeen damaged or lost due to some disease condition. These IPS cells can be derived directly from from adult tissues making it easier to produce and it an provide us with transplants without any risk of immune rejection.

To treat Cystic fibrosis, IPS cells are used to create lung epithelium cells. These epithelium cells form the tissue that line the airways and is the place where the CF affects the lung. Epithelial cells of the lung is the place where CF affects and causes irreversible lung disease and respiratory failure.

IPS cells are cells like fibroblasts. These are collected from the CF patients. They can be repaired with CFTR and reprogrammed to resemble embryonic stem cells (ESC) into fully differentiated cells characteristic of the proximal and distal airways. ESC and IPS cells have identical genes when they are developed from the same donor cells.

These generated epithelial cells then are grown. This contains the delta-508 mutation and G551D mutation which are genes that are mainly responsible in causing CF. Creating IPS cells that contain the entire genome of a Cystic fibrosis patient and direct them to become lung progenitor cells which develop into epithelium. Thus, IPS cells can provide use with a personlaized new tissue to replace the diseased tissue.

gladiator answered 1 year ago

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