Cysteine & Glutathione - 2

[Guest guest]

This is the email I got from Ginger (who was on the ES list). I asked her alot of questions regarding this subject a couple years ago.

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<Hi Ginger,

My name is Qadoshyah Fish, I am 16 years old and am sister to an almost 10 month old little boy with Down Syndrome.>

Hi there. That is an interesting name- I’ve never heard it before!!

I have to commend you on doing this work. My Carmen (now almost 8 years old) has an almost 15-year-old sister who is her best friend and biggest cheerleader. She has been a tremendous influence in Carmen’s life and they share an amazingly special bond.

<I am on the ES list and have been reading the last info you put up about the IL-6 stuff and all that. I have been doing alot of research about the boichemical make-up of DS individuals and the nutritional area involved. My brother is on NuTriVene-D. Anyways, I have one question:

I know that generally Down Syndrome individuals are low in L-Glutathione and high in L-Cysteine. In the Circle of Friends book II on page 104, Dr. Swenson writes,

L-Cysteine. . . Children with DS overproduce this amino acid. The action of certain enzymes on cysteine can produce the toxic product, H2S, or can lead in some circumstances of overproduction of Taurine . . .

How can DS individuals be low in L-Glutathione, yet be high in L-Cysteine, and L-Cysteine be a component of Glutathione? Is that correct that L-Cysteine is a component of L-Glutathione?>

Yes, cysteine is a component of glutathione. Glutathione is a tripeptide, meaning it has 3 amino acids that make it up. These are glycine, cysteine and glutamic acid (aka glutamate). So, to make a complete molecule of glutathione, you need one of each of those 3 amino acids. In most people, the “limiting” amino acid (which means the one you have the least of) is cysteine. That is why most stuff you read about glutathione tells you to supplement cysteine to improve your glutathione levels. That is not the case in DS. Because of the CBS gene being on chromosome 21, and being overexpressed (remember, not all genes that are triplicated are turned on, but this one is!) it is busy “stealing” homocysteine from the SAM cycle to make cysteine. That is why there is too much cysteine and not enough SAM in DS. In DS, one of the other amino acids appears to be the limiting one.

There are also 2 forms of glutathione- the reduced form, GSH, which is the active antioxidant, and the oxidized form GSSG which has to be reactivated by vitamin C. There seems to be some evidence that all forms of glutathione are down in DS, but the real kicker is that since the kids are under extra oxidative stress, more GSH (the active stuff) has to sacrifice itself to become GSSG to rectify the situation. So you can be clinically low in GSH while having plenty of GSSG running around in your body. That is why so little is left for “normal” activity, and why it is so important to take antioxidants, especially vitamin C, to help the body out. So, even if you make enough GSH to begin with, it may not be able to work because it has been deactivated.

There’s a nice, though slightly technical, discussion of glutathione on http://www.thorne.com/altmedrev/fulltext/glut.html. I think the figures may especially helpful. It does not address the special case of DS.

<Is Acetyl-L-Cysteine the same thing as L-Cysteine?>

No, they are chemically different. Acetyl-L-Cysteine is the same as N-acetyl-L-cysteine (NAC). The second name is just more chemically precise- that the acetyl group has been added to the Nitrogen on the amino acid. Information about NAC can be found at http://www.pdrhealth.com/drug_info/nmdrugprofiles/nutsupdrugs/ace_0178.shtml.

I asked Dr. Jill about it a couple of years ago, and she said that NAC will not convert backwards to L-cysteine, so she thought that kids with DS might benefit from it.

There is some interesting information about NAC and how it appears to be anti-inflammatory and may help in autoimmunity. You probably remember my comments that I think that inflammation is a huge issue in DS that we need to deal with. http://www.jautoimdis.com/content/2/1/4

<What is your opinion on using Glutathione Cream? It does contain Cysteine in it as well, which is why I am asking some of this stuff because we were looking into this.>

It would naturally have about 1/3 cysteine in it, but it would be bound to the other 2 amino acids. Kids with DS need GSH, and a transdermal cream seems to do the trick. It is a lot less invasive than doing intravenous GSH, which is your other option. A lot of folks in the autism community are using this stuff. Biochemically, kids with DS and kids with autism are very similar. Eerily so. I believe that is why kids with DS are such sitting ducks for getting autism.

The problem with glutathione is that you can’t get it in orally- the stomach tears it up into amino acids. You need to get it into the body bypassing the stomach. Alpha-lipoic acid is another supplement that you can use to increase glutathione, and that is the approach we take. ALA can cause candida in the gut to flare up, so you have to be sure that you have that in check before you go that route. We use the high-probiotic foods suggested in the Body Ecology Diet to make that happen.

<Another question I have is, if children with DS overproduce the amino acid Cysteine and it can lead to the overproduction of L-Taurine, why is L-Taurine in NuTriVene-D? Is there anything bad about over producing Taurine?>

I have honestly not looked at the taurine angle. I know that some breakdown products of cysteine are toxic, and that is the concern. I am not aware of toxic taurine breakdown products. In fact, from my readings in the autism world, taurine is very helpful in detoxing the liver, so many kids on the spectrum take taurine supplements. I would assume it was put into NVD because kids tested functionally low in it. I’ll go with data over theory any day!!

You might want to contact Dr. Dave and ask him for an opinion and an explanation. He is a very nice, helpful guy with a wonderful sense of humor. Be sure you ask for permission to post his reply, and send it to the list, and if you wouldn’t mind, send me a copy since I am not on the list at the moment due to my crazy life. (We’re starting a farm at the moment, and that absorbs all my free time and then some!!)

<I am just trying to put all the pieces of the puzzle together and make sure I have them all straight.

Thanks,

Qadoshyah

Sister to 10 siblings including boy(DS)/girl twins - Feb. 05>

It is a huge puzzle, and we don’t know what all the pieces are yet. If you would like to get a good description of the SAM cycle, try the book “Methyl Magic” by Craig Cooney. I thought that was a very good, readable treatment of a very complicated process.

HTH.

Oh- you may feel free to post this to the list. I imagine that there are others who might find it interesting.

G