1. Using Cystic Fibrosis as a Model Disease to Teach Biology Sharon Gusky Karen Hunter Northwestern CT Community College

2. Topics Covered in Biology that you can link to Cystic Fibrosis Atoms/Ions Plasma Membrane /Channel Proteins Osmosis and Diffusion Cell structure and Function Chromosomes Protein Synthesis Gene Mutation Mendelian Genetics

3. Atoms and Ions Chloride Ions serves as one of the main electrolytes of the body. represent 70% of the body’s total negative ion content. combines with hydrogen in the stomach to make hydrochloric acid,

4. What Is Cystic Fibrosis? (http://www.cff.org/AboutCF/)http://www.cff.org/AboutCF/ Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system About 30,000 children and adults in the United States (70,000 worldwide) have the disease A defective gene and its protein product cause the body to produce unusually thick, sticky mucus

6. Plasma Membrane/Channel Proteins

7. Osmosis and Diffusion

8. Cell Structure and Function

9. DNA/ Chromosomes

10. Protein Synthesis / Mutations

11. Mendelian Genetics

12. A Cystic Fibrosis WebQuest Use the following web resources to answer the questions and learn more about CF I. An introduction to Cystic Fibrosis http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html a. What is cystic fibrosis(CF)? b. How is it inherited? c. What are the symptoms associated with the disorder? II. The CF protein :Section 1 – Understanding the Problem http://learn.genetics.utah.edu/content/tech/genetherapy/cysticfibrosis/index.html a. What type of mutation causes CF? b. What is the normal role of the CF protein? c. What happens when the protein does not work properly?

13. III. Screening newborns http://www.cff.org/AboutCF/Testing/NewbornScreening/http://www.cff.org/AboutCF/Testing/NewbornScreening/ a. What are the advantageous of screening newborns? b. What, if any, are the disadvantages? c. Do newborns in Connecticut get screened for Cystic Fibrosis? IV. Diagnosing Cystic Fibrosis a. How is CF diagnosed? V. Read the information on the three diagnostic test we will be doing in lab Trysinogen Test http://www.labtestsonline.org/understanding/analytes/trypsinogen/glance.html a. Why is further testing needed if the Trysinogen test is positive? Genetic Testing http://www.labtestsonline.org/understanding/analytes/cf_gene/glance.html a. What chromosome is the CF gene on? b. What type of mutation causes CF? Sweat Test http://www.labtestsonline.org/understanding/analytes/sweat_chloride/glance.html a. Why do you think the sweat test is considered the confirming test for CF?

15. Guthrie Card and Patient History

16. Positive Control –Negative Control – 4 Patients

17. PCR

18. Sweat Test