Bronchospheres

So, as I was searching for what to talk to y’all about this week, I came across an article titled “CReM Scientists Turn Human IPS Cells into Lung Cells”. I found this very interesting, so I clicked on it, the first thing I noticed on the page was ““Bronchospheres” may pave way for personalized cystic fibrosis treatments”. Before I start discussing this cool find, let me first tell you a little about cystic fibrosis.

The majority of my blog is going to focus on the article I found, but you first need to know a little background on cystic fibrosis. Cystic fibrosis is an awful genetic disease that is caused from mutations. These mutations are in the CFTR gene. This gene is responsible for sending instructions for making a protein called the cystic fibrosis transmembrane conductance regulator. This protein is responsible for the functioning of the cells that produce mucus, sweat, saliva, tears, and digestive enzymes. The mutation in this gene causes numerous things including abnormal function of the lung and pancreas. For most patients with this mutation, their lungs begin to produce a really thick mucus. This leads to infection, inflammation, and most of the time, lung failure. Unfortunately, most patients who suffer from cystic fibrosis do not get better because currently there is no cure.

Just in case you did not know, lungs and other organs start out as clumps of stem cells, and then the cells get organized and develop into functioning organs. There are scientists who specialize in the study of lungs and lung diseases. These scientists have tried to study the process of lung development in detail to determine where the development goes wrong. Scientists at Boston University’s Center for Regenerative Medicine (CReM) have discovered two findings that help with understanding the process of lung development. These two findings included: “the ability to grow and purify the earliest lung progenitors that emerge from human stem cells, and the ability to differentiate these cells into tiny “bronchospheres” that model cystic fibrosis”.  The researchers hope that these findings and results will lead to “personalized medicine” for patients who suffer from cystic fibrosis.

This long process started with the discovery of induced pluripotent stem cells, or iPSCs that Shinya Yamanaka made. Shinya Yamanaka found a way to take any cell in the human body and reprogram it into a stem cell that has the ability to grow into any organ. CReM scientist uses these cells for their experiments. For years, there were issues with using iPSCs. The resulting lung cells grow with many other cells including: liver cells and intestinal cells. Finally, through many experiments, scientists were able to isolate just lung cells without any other cells.  They called these cells “organoids,” which were smaller versions of the organ. According to Katherine McCauley, this discovery meant that scientists could finally look at specific diseases, like cystic fibrosis.

McCauley was able to locate a pathway called Wnt that was known to be important in lung development. She turned the pathway off, and guided the immature lung cells into becoming airway cells. Next the cells were grown into tiny calls and called “bronchospheres.” The goal of these “bronchospheres” or collection of specific cells was to be able to study and learn more about lung diseases like cystic fibrosis. McCauley obtained two cell lines from a patient with cystic fibrosis. The cell lines were cells that contained the CFTR mutation that caused the disease had been corrected, and one in which it hadn’t. These two lines were then grown into bronchospheres. She then ran an experiment where she used a drug that should cause bronchosphere made of functioning cells to fill with fluid. Her experiment was a success as she saw the “fixed” bronchospheres swell, and the cystic fibrosis spheres did not swell.

This find from McCauley was huge because eventually scientist will be able to extract patient’s cells and test hundreds of drugs on these cells to see and understand how the patient is going to respond before treatment is performed. This is actually pretty awesome because, in the long run, it will help many patients get the right treatment instead of just guessing and seeing what works.

Sources:

https://ghr.nlm.nih.gov/gene/CFTR

https://www.bu.edu/research/articles/crem-scientists-turn-ips-cells-into-lung-cells/