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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?

Very salty-tasting skin, persistent coughing, lung infections, wheezing or shortness of breath, poor growth, and frequent greasy, bulky stools.

2. How common is this disorder?

There are about 1,000 new cases of CF each year, 70% of paitents are diagnosed by age two, 45% of the CF population is age 18 or older.

3. How is cystic fibrosis diagnosed?

Genetic testing or new born screening

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

The child must inherit one copy of the defective CF gene from each parent.  No.

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.    Normally, the movements of ions brings water to the surface of the airway and keeps the mucus moist.

2. What happens to this protein in CF patients and what are the consequences for the health of these individuals? The chloride channel is blocked therefore there is no movement of chloride ions into the mucus. There is no need for water to move towards the surface and the mucus dries out. There fore the paitents lungs do not function correctly.

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 “Treatment” and “Self-care” sections to answer the following questions:

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

Medications, Therapy, and surgeries.

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

 Drinks lots of fluids, Excercise, eliminate smoke.