BSHRM-1

1:30-3:00 TTH FOOD-CHEM

What genetic disorder does Lorenzo have? Adrenoleukodystrophy (ALD) It is a progressive degenerative myelin disorder, meaning that myelin, the "insulation" around nerves, breaks down over time. Without myelin, nerves can't function normally, or at all. Unfortunately, the body can't grow replacement myelin, so the disorder is progressive. What Causes ALD, and Who Develops It? ALD is an inherited recessive genetic disorder linked to the X chromosome. Because of the way genetic inheritance works, only boys have the most severe form of ALD. The disorder leaves the body unable to break down big fat molecules, either ones the body makes itself or ones that enter the body through food. Research has shown that this is most likely due to a carrier protein that fails to work correctly and carry the fat molecules to where they would be broken down. The fat molecules build up and clog up cells, and hurt nerve cells in the brain and spinal cord. ALD affects about one in 20,000 males. One gene, called ABCD1, has been identified as being associated with ALD. Genetic testing can be done to see if a woman has the defective gene. This way, a woman who may have inherited an abnormal gene will know for certain whether or not she has it (is a carrier) and could pass it on to her children. What are the components of Lorenzos Oil? Triolein is a triglyceride and unsaturated fat formed from oleic acid. Triolein is used in Semipermeable membrane devices (SPMD) to collect organic compounds in form of a passive sampling device in water analysis.[1] the collecting compound triolein is placed in a tube and set up in a cage like container into the water system (such as streams, rivers and other water reservoirs). Triolein is also a component of Lorenzo's oil.

What is the mode of action of Lorenzos Oil in preventing and treating ALD in the story? This mixture of fatty acids reduces the levels of very long chain fatty acids (VLCFA) known to cause ALD. It does so by competitively inhibiting the enzyme that forms VLCFAs. What significant learnings did you realize from the film? My significant learnings about Lorenzos life realize what life important even if he knows he cant be cured for such disorder, he still have the courage to test all the possibilities just to cure his disorder and also the support of the parents of Lorenzo did not give-up easily.

What life realities does the film portray? The life realities which the film portray the complicated rare disorder which 1 out of 10 kids would been affected on such reactions from the body.