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By: Clara Alonso-Stepanova, Anisa Guedira and Ani Marvenova

Academic Integrity Statement:
We pledge to be honest and fair. We have neither given nor received any unauthorized aid on this assignment .

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G-protein

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Calcium and cAMP

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Drugs such as dornase alfa (Pulmozyme), inhaled tobramycin (TOBI), and ivacaftor (Kalydeco) have been developed to fight Cystic Fibrosis.

Pre-clinical work is funded to help develop drugs that are effective and safe for consumption (animal trials are used in order to ensure safety).

As of 2017, the FDA has approved ivacaftor (Symdecko) for people with two copies of F508del as well as people with at least one of the 26 mutations.

Citations:
https://www.cff.org/Research/About-Our-Research/Research-Milestones/

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Citation: http://pathwaymaps.com/maps/2269

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Cystic Fibrosis happens as a result of a defect in the cystic fibrosis transmembrane conductance regulator. This gene controls the movement of water and salt in and out of the body's cells. A sudden mutation or change in the gene causes the mucus to become thicker and stickier than in people without the mutation. CFTR is involved in the diffusion of chloride ions.

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In a normal CFTR signaling pathway, calcium is actively imported from the cytosol and into the ER, making the calcium concentration higher in the ER than the cytosol. Adenosine is the reporter molecule on the ASL. In addition the CFTR is opened by ATP binding to the NBD2 site of the channel. A problem with posttranslational modifications causes the mutation.

In CF the F508del-CFTR protein has an improper folding and is abnormally retained in the endoplasmic reticulum. The abnormal channel breaks down shortly after it is made, so it never reaches the cell membrane to transport chloride ions.

The CFTR gene provides instructions for making a channel that transports negatively charged  chloride ions into and out of cells. Chloride is a component of sodium chloride, a common salt found in sweat. Chloride also has important functions in cells; for example, the flow of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus.

Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes.

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What is CF?
Cystic Fibrosis is an inherited disorder that causes severe damage to the lungs , digestive system and other organs in the body. It has an autosomal recessive inheritance.

What causes CF?
It is a deletion of one amino acid at position 508.The thick, sticky mucus that is associated with cystic fibrosis blocks the passageways that carry the air into and out of the lungs.

Symptoms:

Digestive Problems

Greasy, foul-smelling stools

Constipation

Nausea

Swollen abdomen

Loss of appetite

Poor weight gain in children

Delayed growth in children.