Lorenzo’s Oil Obectives: To demonstrate understanding of…..  How Lorenzo inherited the disease through genetic inheritance.  How a disease is the result of a malfunctioning system, organ, and cell.   The importance of proteins to the normal breakdown of fatty acids. The implications of abnormal metabolic functioning in ALD patients. Fatty acid manipulation as a therapeutic treatment in ALD patients using Lorenzo’s oil. Ethical implications demonstrated by the Lorenzo Odone story.  

How Lorenzo inherited the disease through genetic inheritance. Lorenzo’s Oil Obectives: To demonstrate understanding of…..  How Lorenzo inherited the disease through genetic inheritance. What is a sex-linked gene? Why do sex linked disorders affect males more often? Which parent passed the defective gene to Lorenzo? Why don’t Lorenzo’s parents suffer ALD? What was the defective gene responsible for? (What was it suppose to code for?) Illustrate how Lorenzo inherited ALD using a punnett square. See 9.21 & 9.22 in text for additional help.

Lorenzo’s Oil 2.  How is ALD the result of a malfunctioning system, organ, and cellular level? Organ System Level: What organ system is failing? Why is it failing? Organ Level: What organ is failing? Why is it failing? Cellular Level: What is happening at a cellular level that causes cell destruction? Why is this happening?

3. The importance of proteins to the normal breakdown of fatty acids. Lorenzo’s Oil 3. The importance of proteins to the normal breakdown of fatty acids. What type of proteins are involved in ALD? How are enzymes involved in ALD? How does competitive inhibition in enzyme activity help reduce the biosynthesis of VLCFA?

The implications of abnormal metabolic functioning in ALD patients. Lorenzo’s Oil The implications of abnormal metabolic functioning in ALD patients. Metabolic Pathway: A series of chemical rxns that either builds a complex molecule or breaks down a complex molecule into smaller units. Describe ALD in terms of a malfunctioning metabolic pathway. Explain why diet alone cannot help ALD patients in terms of biosynthesis?

Lorenzo’s Oil Fatty acid manipulation as a therapeutic treatment in ALD patients using Lorenzo’s oil. Lorenzo’s oil is a mixture of oleic acid (from olive oil) and erucic acid (extracted from rapeseeds). How are these oils used as a treatment? Discuss answer in terms of competitive inhibition in enzyme activity and biosynthesis. Why don’t these oils harm ALD patients? How do these oils differ structurally from the fatty acids that build up in ALD patients?

6. Ethical implications demonstrated by the Lorenzo Odone story. Lorenzo’s Oil 6. Ethical implications demonstrated by the Lorenzo Odone story. The process of finding medical treatments for people (clinical trials) often puts sound scientific processes (repeating trials; having control groups; taking time for peer review) against human morales (helping those who suffer). How is this highlighted in Lorenzo’s story? Be able to give specific examples.

Long Chain Fatty Acids & ALD VISIT this site http://clasfaculty.ucdenver.edu/bstith/loren.htm The Myelin Project Play slide show http://www.myelin.org/resources/toolsforteachers.html http://www.myelin.org/ - Link to Lorenzo’s Oil