Sickle Cell Anemia-- Understanding the Molecular Biology. A Biology Workbench Tutorial
Brent Palmer
(bopalmer@uiuc.edu)
(ready to use)
Coauthors
ASK
Partner Projects
| Biology Student Workbench |
Subject Areas
| Education, Health, Information Science, Science |
Grade Levels
| 9, 10, 11, 12, Undergraduate, Graduate |
Unit Keywords
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LIS350GK, 350GK, 350 GK, GK-12, Biology Workbench, Biology Student Workbench, biology, laboratory,bioinformatics, protein |
Rationale of the Unit
Sickle Cell Anemia is a genetic disorder that is characterized by the formation of hard, sticky, sickle-shaped red blood cells, in contrast to the biconcave-shaped red blood cells (RBCs) found in “normal” individuals. This tutorial looks specifically at the mutation in hemoglobin that is the cause of this painful disease.
The purpose of the tutorial is two-fold. Firstly, you will learn how to search databases for DNA and protein sequences, and how to align and manipulate these sequences. You will also learn how to view 3-dimensional structures of protein molecules using sequences that you find in the first part of this tutorial. Secondly, you will learn about the molecular biology of an extremely common and painful disease, sickle cell anemia. To carry out this tutorial you will use two very important Web-based interfaces, the Biology Workbench and Protein Explorer. The Biology Workbench was initially designed for researchers to search for and analyze sequences of nucleic acids and proteins. In order to become familiar with this comprehensive program, we have outlined the basic tools of the Workbench in the following tutorial. The Protein Explorer is a 3D-modeling program. You will learn how to use the Protein Explorer to see where the sickle cell anemia mutation occurs and how it affects the structure of the hemoglobin protein. |
Background and Resources
The main resource used to create this tutorial was the Biology Workbench (http://workbench.sdsc.edu ) and the Protein Explorer (http://molvis.sdsc.edu/protexpl/frntdoor.htm ). Note that there are browser requirements:
Windows:
Netscape Communicator 4.7X with Chime 2.0.3 or later(Recommended)
Netscape Navigator 4.08 with Chime 2.0.3 or later.
Internet Explorer Version 5.5, Update Version SP2 with Chime 2.6 SP3 or later.
Internet Explorer version 6.
Macintosh:
Netscape Communicator 4.7X with Chime 2.0a or later(Recommended)
Netscape Navigator 4.08 with Chime 2.0a or later.
(Not Internet Explorer.)
Other references cited in the tutorial:
Davies SC, Oni L. Management of Patients with Sickle Cell Disease. BMJ. 1997 Sept. 13; 315(7109): 656-60.
Steinberg MH. Pathophysiology of Sickle Cell Disease. Baillieres Cinical Haematology. 1998 Mar: 11(1):163-84.
World Wide Web.. Sickle Cell Anemia. BluePrint for Health Visited 10/10/00. http://blueprint.bluecrossmn.com/topic/sickle.
World Wide Web. Sickle Cell Information-Clinician Summary. The Sickle Cell Information Center. Visited 10/10/00. http://www.emory.edu/PEDS/SICKLE/prod05.htm
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Activities and Open-ended problems
The current form of the tutorial can be found at the Biology Student Workbench:
http://peptide.ncsa.uiuc.edu/tutorials_current/Sickle_Cell_Anemia/SC2001/index.html The tutorial guides the investigator through the process of logging onto the Biology Workbench, finding and importing the normal and mutant protein sequences, aligning and comparing the sequences and viewing the 3-D structure with Protein Explorer (note Browser requirements above).
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Assessment, Related Questions, and Story of the Unit
As a follow-up, there is a tutorial on Familial Cholesterolemia that uses a few more "advanced" bioinformatics tools. In sickle-cell anemia the protein defect is dramatic, as the mutant protein has a dramatically altered shape. In Familial Cholesterolemia, the effect of mutation on the protein structure is small, requiring use of more sophisticated tools to begin to see the difference between normal and mutant protein.
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