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?
Salty tasting skin, persistent coughing; at times with phlegm, frequent lung infections, wheezing or shortness of breath, poor growth/weight gain in spite of good appetite, and frequent greasy, bulky stools or difficulty in bowel movements.
2. How common is this disorder?
30,000 people in the U.S. 70,000 people in the world
3. How is cystic fibrosis diagnosed?
Many tests such as the sweat test, newborn screening, and the genetic carrying test.
4. How is cystic fibrosis inherited? Does everyone who has a mutant gene for the protein have cystic fibrosis?
By getting the gene, 10 million people who live in the U.S. have a symptomless version on the defective 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.
Normally, the movements of ions brings water to the surface of the airway and keeps the mucus moist. The defective gene acts to block the channels, which causes the mucus to dry out. It is then difficult for a person to shift the mucus, which then becomes prone to infection by bacteria.
2. What happens to this protein in CF patients and what are the consequences for the health of these individuals?
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.
Antibiotics, Mucus-thinning drugs, Bronchodilators
2. Discuss at least 3 ways for parents to help their children who have cystic fibrosis.
Chest clapper, Inflatable vest, Breathing devices
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?
Salty tasting skin, persistent coughing; at times with phlegm, frequent lung infections, wheezing or shortness of breath, poor growth/weight gain in spite of good appetite, and frequent greasy, bulky stools or difficulty in bowel movements.
2. How common is this disorder?
30,000 people in the U.S. 70,000 people in the world
3. How is cystic fibrosis diagnosed?
Many tests such as the sweat test, newborn screening, and the genetic carrying test.
4. How is cystic fibrosis inherited? Does everyone who has a mutant gene for the protein have cystic fibrosis?
By getting the gene, 10 million people who live in the U.S. have a symptomless version on the defective 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.
Normally, the movements of ions brings water to the surface of the airway and keeps the mucus moist. The defective gene acts to block the channels, which causes the mucus to dry out. It is then difficult for a person to shift the mucus, which then becomes prone to infection by bacteria.
2. What happens to this protein in CF patients and what are the consequences for the health of these individuals?
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.
Antibiotics, Mucus-thinning drugs, Bronchodilators
2. Discuss at least 3 ways for parents to help their children who have cystic fibrosis.
Chest clapper, Inflatable vest, Breathing devices
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