Cystic fibrosis

2023 - 3 - 27

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Image courtesy of "Cystic Fibrosis News Today"

Study delves into how CFTR changes its shape to work as intended |... (Cystic Fibrosis News Today)

Small changes in the shape of CFTR, the protein defective in CF, inside the cell opened its gate outside the cell, allowing chloride ion flow.

“There is potential here to help cystic fibrosis patients by learning about the structure and behavior of CFTR,” said Jesper Levring, the study’s first author with Chen’s lab. “Through this collaboration, we had the opportunity to really dial into the relationship between structure and function,” said Blanchard, the study’s other co-lead researcher. Notably, this mutation also impaired NBD dimerization despite being located far from the NBDs in space. With activation, the two NBDs came together — or dimerized — and the channel opened. Data showed the G551D mutation affected the shape of the ATP binding site, which impacted NBD dimerization and disrupted the link between NBD and the gate. Researchers showed that ATP-dependent NBD dimerization alone was not enough to open the gate, as previously proposed. The transmembrane domains span the cell membrane to form the channel pore and gate, leading outside the cell. CFTR is a protein embedded in the cell membrane that transports negatively charged chloride ions in and out of cells. In response to signals within the cell, enzymes first modify CFTR’s regulatory domain by attaching a phosphate molecule (phosphorylation), which acts like an “on” switch. “Every individual method has limitations, so you can have good data but still not have the answers.” Inside the cell are the regulatory and nucleotide-binding domains (NBDs). Before activation, the channel was closed and the two NBDs were far apart in space.

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Image courtesy of "Cystic Fibrosis News Today"

Saying farewell to my column, but not my cystic fibrosis community... (Cystic Fibrosis News Today)

In her final column for CF News Today, Nicole Kohr shares what she's learned and thanks her cystic fibrosis community for their support.

[cystic fibrosis community](https://cysticfibrosisnewstoday.com/columns/tarzan-strangers-like-me-open-letter-cf-community/) and chronic illness as a whole. [Cystic Fibrosis News Today](http://cysticfibrosisnewstoday.com/) is strictly a news and information website about the disease. If you haven’t read any of my columns, I’d love it if you dipped your toes into the world of “ [Lung, Scrappy, and Hungry](https://cysticfibrosisnewstoday.com/lung-scrappy-and-hungry-nicole-kohr/)!” I bonded with fellow transplant recipients after “ [‘Changing My Major (Organs): A Chronicle of Bilateral Lung Transplant](https://cysticfibrosisnewstoday.com/columns/bilateral-lung-transplant-changing-major-organs/).'” The community just started resharing my [Make-A-Wish](https://cysticfibrosisnewstoday.com/columns/big-fun-my-make-a-wish-birthday-party/) column after the [recent announcement](https://cysticfibrosisnewstoday.com/columns/problem-make-a-wishs-new-policy-children-with-cf/) that many CF patients will no longer qualify for a wish. It does not provide medical advice, [diagnosis](http://cysticfibrosisnewstoday.com/diagnosis-of-cystic-fibrosis/), or [treatment](https://cysticfibrosisnewstoday.com/approved-treatments-for-cystic-fibrosis/). [Learning to Embrace My Poor Unfortunate Holes](https://cysticfibrosisnewstoday.com/columns/scars-poor-unfortunate-holes/),” a lyrical pun that still makes me giggle. [Titanic](https://cysticfibrosisnewstoday.com/columns/my-fight-antibiotics-cystic-fibrosis-patient/)” series landed me a speaking gig about antibiotic resistance. Luckily, there are [many talented writers](https://cysticfibrosisnewstoday.com/category/columns/) who can pick up where I left off. [Cystic Fibrosis News Today](https://cysticfibrosisnewstoday.com/) to have such a positive and lasting impact on my life. So many of my close friends were struggling with their self-esteem at the time (as it related to their ports and feeding tubes), and that column opened a therapeutic dialogue between us. I became a foster parent and elected to write twice a month instead of once a week. With that said, I can’t think of a stronger way to depart than with the assistance of two strong writers: Alexander Hamilton and Lin-Manuel Miranda.

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