[amit]

I read that in one type of CMT - the reason for the death of the nerves is clusters of mitochondias. My question is - if in this type of CMT (I think type 2, but not sure) - is it wise to take Q10 or acetyl-L-carnitine, which activated the mitochondia?

[mrsD]

We cannot live without our mitochondria.

One form of CMT has been shown to have a defect in the mitochondrial functions of energy production.

This is the actual quote:

The disorder is caused by the absence of proteins that are essential for normal function of the nerves due to errors in the genes coding these molecules. The absence of these chemical substances gives rise to dysfunction either in the axon or the myelin sheath of the nerve cell. Most of the mutations identified result in disrupted myelin production, however a small proportion of mutations occur in gene MFN2, which doesn't seem to have anything to do with myelin. Instead MFN2 controls behaviour of mitochondria. Recent research showed that the mutated MFN2 causes mitochondria to form large clusters. In nerve cells these large clusters of mitochondria failed to travel down the axon towards the synapses. It is suggested these mitochondria clots make the synapses fail, resulting in CMT disease.[2]

from http://en.wikipedia.org/wiki/Charcot...-Tooth_disease

CMT is a very complex hereditary disorder.
This means that certain genes were inherited that disrupt the functions of the mitochondria in CMT patients. And that disruption varies according to the genetic problem. There are many types of CMT.

But mitochondria also exist in everyone else, who do not have CMT. Damage to these can come from the environment, vaccines and drugs. Antibiotics that kill bacteria that cause infections can also kill/damage our mitochondria..since mitochondria are evolutionary decendants of bacteria, that animals incorporated into cells to create energy.
People with frank mito disorders which are also hereditary, are given acetyl carnitine and CoQ-10 as treatments.

I cannot say about CMT patients...since they have problems with respiration of the mitochondria in some types. (Vit C is being trialed for those specific ones), what all treatments may do. I don't think anyone knows this yet. The research is so far only focusing on Vit C for specific types.

So for now, where we are at the beginning of finding answers to chronic illnesses suspected of having some mito failure features, we can only suppose supporting the mitochondria would be helpful.

Carnitine and CoQ-10 are made in our bodies as natural substances. Supplementing them just raises their levels. Some drugs deplete CoQ-10...statins, diuretics, beta blockers are examples.

I think with PN, it is worth trying things that have a very negligible side effect profile, but promise to improve functioning.

Here is another biological example:
Down's syndrome patients (genetic disorder) have a higher rate of leukemia and other blood system disorders. But not all people who develop leukemia, or multiple myeloma, have Down's syndrome.

If you think you have CMT...you should get tested for it, IMO.

BTW... carnitine and CoQ-10 work by transporting nutrients across the mitochondrial wall. Carnitine transports free fatty acids when glucose is low.
http://en.wikipedia.org/wiki/Carnitine

CoQ-10:
http://en.wikipedia.org/wiki/Coenzyme_Q10

and:

The conversion of energy from carbohydrates and fats to adenosine triposphate (ATP), the form of energy used by cells, requires the presence of coenzyme Q in the inner mitochondrial membrane. As part of the mitochondrial electron transport chain, coenzyme Q accepts electrons from reducing equivalents generated during fatty acid and glucose metabolism and then transfers them to electron acceptors. At the same time, coenzyme Q transfers protons outside the inner mitochondrial membrane, creating a proton gradient across that membrane. The energy released when the protons flow back into the mitochondrial interior is used to form ATP (2).

from http://lpi.oregonstate.edu/infocenter/othernuts/coq10/

[Kitt]

If you do have a type of CMT Amit, it would help to know what type. Then you can go from there.

CMT2A2 is where the linkage or gene is mentioned. CMT2's are autosomal dominant axonal/neuronal.

The Ascorbic Acid (Vitamin C) trials are for those who have CMT1A. There have been some that have been completed in other countries and I believe about four or so trials ungoing in the U.S. now. Hopefully, something will come out of them for people who have CMT1A.

There are many types of CMT and it is complicated. There are 50 kinds identified so far and there is no end in site. They can do DNA blood testing for 22 + types. Just because the test does not turn up for one of these types does not mean that you do not have CMT.

Again, it is very complicated and there is no easy fix. CMT is far too complicated with all the types that are known and those that are not known yet for any lay person to get very far into it. That also includes those of us who are living everyday of our life with one type of CMT or another type of CMT. And it also includes any neurologists/doctors who know near nothing about it. You need an expert in CMT to guide you thru your particular type if indeed you have it.

The most common type is CMT1A.