Cystic Fibrosis Webquest

Along with the many other webquests we have done, this one was about cystic fibrosis.  A genetic disease that messes with the carrier proteins in your lungs.  Below are the answers and links to websites along with the webquest.

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CYSTIC FIBROSIS – A DISORDER OF MEMBRANE TRANSPORT

A great variety of proteins have roles in moving molecules and ions across cell membranes. Passive transport proteins permit certain substances to diffuse down concentration gradients by moving through the protein’s interior. Active transport proteins use ATP energy to pump substances across the membrane against their concentration gradients. To investigate the importance of transport proteins, we will consider the effects of cystic fibrosis, a genetic disorder in which there is a defect in a transport protein..

Activity

Part 1.

In this part of the activity you will visit the Cystic Fibrosis Foundation’s web site to learn about the causes and symptoms of cystic fibrosis.

Use your browser to go to http://www.cff.org/home/

Use the information provided in the “About cystic fibrosis” section to answer the following questions:

1. What are the signs and symptoms of cystic fibrosis?

Signs and symptoms of cystic fibrosis are salty-tasting skin, coughing, frequent lung infections like pneumonia and bronchitis, short breath, and poor growth habits.

2. How common is this disorder?

Cystic fibrosis is not the most common disease because both parents have to be carriers to contract it.  About 30,000 people in the U.S. have it.

3. How is cystic fibrosis diagnosed?

You can diagnose cystic fibrosis with a sweat carrier test or by a genetic test.

4. How is cystic fibrosis inherited? Does everyone who has a mutant gene for the protein have cystic fibrosis?

Cystic fibrosis is inherited when both parents carry the defective gene.  No, not everyone who has the mutant gene has cystic fibrosis.  You can be a carrier of the gene.  You only inherit cystic fibrosis when both parents are carriers of the gene.

Part 2.

In this part of the activity you will read an article to learn more about cystic fibrosis.

Use your browser to go to:

http://resources.schoolscience.co.uk/MRC/3/page3.html

Use the information in this article to answer the following questions:

1. Explain the normal function of the protein that is defective in cystic fibrosis.

The protein is called the CF transmembrane regulator.  The channel is first closed as phosphate groups build up.  A bound ATP is hydrolysed to the phosphate group.  The shape of the channel changes and opens the channel.

2. What happens to this protein in CF patients and what are the consequences for the health of these individuals?

The protein doesn’t allow ions to pass through channel linings.  So, water can’t be moved in this way to keep the mucus moist.  The mucus becomes dry and it makes it hard for the mucus to move.  Patients are more prone to bacterial infections.

Part 3.

In this part of the activity you will read about how cystic fibrosis is treated.

Use your browser to go to:

http://www.mayoclinic.com/health/cystic-fibrosis/DS00287

Use the information in the different sections of the article to answer the following questions:

1. Explain at least 3 treatments for the symptoms of cystic fibrosis.

Three symptom treatments are oxygen therapy, chest physical therapy, and a lung transplant.

2. Discuss at least 3 ways for parents to help their children who have cystic fibrosis.

Parents can help their children by paying attention to nutrition and fluid intake, keeping immunizations up to date, and eliminating smoke.”