Lorenzo - PowerPoint PPT Presentation

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Lorenzo

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Lorenzo s Oil Adrenoleukodystrophy (ALD) ALD patients are unable to breakdown very long chain saturated fatty acids (VLCSFA). Build up of VLCSFA are toxic to myelin ... – PowerPoint PPT presentation

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Title: Lorenzo


1
Lorenzos Oil
2
Adrenoleukodystrophy (ALD)
  • ALD patients are unable to breakdown very long
    chain saturated fatty acids (VLCSFA).
  • Build up of VLCSFA are toxic to myelin of nerves
  • Breakdown of nerves results in symptoms of ALD
    including loss of motor function, loss of speech,
    and seizures

3
What are fatty acids?
Saturated fatty acids
Unsaturated fatty acids
4
Cell Biology
5
Summary of ALD
Saturated Fat
I make very long chain fats by adding carbon
atoms to the ends of BOTH types of fats in the ER
Unsaturated Fat
6
Summary of ALD
When I am working right, I breakdown excess fats
in the peroxisome
7
Summary of ALD
8
Summary of ALD
9
Summary of ALD
10
How Can We Fix it?
11
Enzyme Function
substrate
product
ENZYME
12
Competetive Inhibition
substrate
product
Competetive inhibitor
ENZYME
13
Lorenzos Oil
Less saturated fats in the diet
Lorenzos Oil
14
Competitive Inhibition
I am so busy working with all the unsaturated
fats, that I dont have time to add carbon
molecules to the saturated fats
Less saturated fats in the diet
Lorenzos Oil
15
Too many saturated fats are BAD for our nerves
Healthy nerve
Unhealthy nerve
16
Unmyelinated Nerves Dont work
17
Is Lorenzos Oil a treatment or a cure?
  • Lorenzo is still alive today paralyzed by ALD
  • His remarkable survival is due to his parents'
    refusal to accept the doctors' grim diagnosis.
  • With no scientific training, they set out to find
    a cure and in 1986 it seemed they had succeeded.

http//news.bbc.co.uk/2/hi/health/3907559.stm
18
Nerve Function
  • Nerves send electrical signals to and from the
    brain
  • They work similar to electrical wiring

19
Myelineated Nerve
  • Myelin sheaths on nerves are made of fats and
    help to speed up nerve impulses
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