Lorenzo’s OilLorenzo’s Oil

Adrenoleukodystrophy Adrenoleukodystrophy (ALD)(ALD)

ALD patients are unable to ALD patients are unable to breakdown very long chain saturated breakdown very long chain saturated fatty acids (VLCSFA).fatty acids (VLCSFA).

Build up of VLCSFA are toxic to Build up of VLCSFA are toxic to myelin of nervesmyelin of nerves

Breakdown of nerves results in Breakdown of nerves results in symptoms of ALD including loss of symptoms of ALD including loss of motor function, loss of speech, and motor function, loss of speech, and seizuresseizures

What are fatty acids?What are fatty acids?Saturated fatty acids Unsaturated fatty acids

Cell BiologyCell Biology

Summary of ALDSummary of ALD

Saturated Fat

Unsaturated Fat

I make very long chain fats by adding carbon atoms tothe ends of BOTH types offats in the ER

Summary of ALDSummary of ALD

When I am working right, I breakdown excess fats in the peroxisome

Summary of ALDSummary of ALD

Summary of ALDSummary of ALD

Summary of ALDSummary of ALD

PROBLEM

NO PROBLEM

How Can We Fix it?How Can We Fix it?

BAD

GOOD

Enzyme FunctionEnzyme Function

ENZYME

substrateproduct

Competetive InhibitionCompetetive Inhibition

ENZYME

Competetiveinhibitor

substrate product

Lorenzo’s OilLorenzo’s Oil

GOOD

Less saturated fats in the diet

Lorenzo’s Oil

Competitive InhibitionCompetitive Inhibition

Less saturated fats in the diet

I am so busy working with all the unsaturated fats, that I don’t have time to add carbon molecules to the saturated fats

Lorenzo’s Oil GOOD

Too many saturated fats are Too many saturated fats are BAD for our nervesBAD for our nerves

Healthy nerve

Unhealthy nerve

Unmyelinated Nerves Don’t Unmyelinated Nerves Don’t workwork

Is Lorenzo’s Oil a treatment or Is Lorenzo’s Oil a treatment or a cure?a cure?

Lorenzo is still alive today paralyzed by ALDLorenzo is still alive today paralyzed by ALD His remarkable survival is due to his parents' His remarkable survival is due to his parents'

refusal to accept the doctors' grim diagnosis.refusal to accept the doctors' grim diagnosis. With no scientific training, they set out to find With no scientific training, they set out to find

a cure and in 1986 it seemed they had a cure and in 1986 it seemed they had succeeded. succeeded.

http://news.bbc.co.uk/2/hi/health/3907559.stm

Nerve FunctionNerve Function

Nerves send Nerves send electrical signals to electrical signals to and from the brainand from the brain

They work similar to They work similar to electrical wiringelectrical wiring

Myelineated NerveMyelineated Nerve

Myelin sheaths Myelin sheaths on nerves are on nerves are made of fats made of fats and help to and help to speed up nerve speed up nerve impulsesimpulses