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Posted
Okay---
I don't have enough evidence so far to go on because I seem to be one of the few who have had my homocysteine levels tested from the dysautonomia site.
But here are the connections---
high homocysteine breaks down connective tissue, leading to "double jointed-ness" or hypermobility (some people having it worse than others/ varying degrees) and "growing pains" as children, a few people even having early-onset arthritis.

Homocysteine, because it breaks down connective tissue, breaks down the lining of veins, arteries, damages the vascular system. Breaks down collagen, elastin, fibrillin. This would attribute to Orthostatic Intolerance? ....Possibly a lower limb Atherosclerosis and as progression of the disease also effects the arteries around the heart???

Homocysteine is a major part of the Methylation Cycle and the Transsulfuration Pathway. This is the "sulfur atom" I talked about months ago that must be a problem. Somehow this screw up makes patients more likely to be susceptible to severe "allergic reactions" from Sulfites and Sulpha drugs. Probably because they already have too many sulphur atoms that need to be methylated.

I would even suggest that while some are simply not converting enough Homocysteine to Methionine (and thus low on Methionine), others have too much Methionine and not enough Glycine being formed (whether there is a lack of B6 and/or Serine may be the problem). Not enough Glycine would further screw up production of connective tissue by reducing collagen. Also--- perhaps people with the severe reactions to sulfites would perhaps have too much sulfite in the system and not enough Glycine (which would be another path that homocysteine could form into). Also, lack of Glycine would attribute to brittle dry hair.

Of course this would not be ALL dysautonomia patients, however it would explain many. However, if anything it also supports the idea that people with Dysautonomia syndromes have some sort of a malfunction with their methylation cycle. For others it may be somewhere else in the cycle, and for many there may be other parts besides Homocysteine that is screwing up. Of course it should be noted that once there is a breakdown in one part of the cycle, the whole thing falls apart.

Take care,
Gen
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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Very interesting... You go girl. Maybe you should go into medical research instead of film Wink
 
Posts: 164 | Registered: February 14, 2008Edit or Delete MessageReport This Post
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I'm too tempermental. I'm writing a novel, that I can turn into a film, and include all the a-hole stupid doctors I had ---to throw in the face of the medical community just how inept so many are. Then I can go on Oprah. Wink Smiler Then Oprah will tell the whole freakin world, and when Oprah speaks, people listen. LOL Wink
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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LOL! What a great idea! You've got it all figured out, don't you! Rock on!
 
Posts: 164 | Registered: February 14, 2008Edit or Delete MessageReport This Post
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You are right on nitekitty! Are you sure you're not a famous biologist in disguise?

Homocystinuria, also known as Cystathionine beta synthase deficiency, is an inherited disorder of the metabolism of the amino acid methionine, often involving cystathionine beta synthase. It is an inherited autosomal recessive trait, which means a child needs to inherit the defective gene from both parents to be affected.

This defect leads to a multisystemic disorder of the connective tissue, muscles, CNS, and cardiovascular system. Homocystinuria represents a group of hereditary metabolic disorders characterized by an accumulation of homocysteine in the serum and an increased excretion of homocysteine in the urine. Infants appear to be normal and early symptoms, if any are present, are vague.

http://emedicine.com/neuro/topic578.htm
 
Posts: 297 | Registered: March 17, 2008Edit or Delete MessageReport This Post
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This is fascinating... I love that people are finally starting to make biological and physiological connections within the diagnosis of Dysaut... It's also so exciting that so many of us are doing so much research on our own, and taking responsibility and initiative in our OWN health care! Yay!!! Good for you guys!!!
 
Posts: 114 | Location: Washington, DC | Registered: January 28, 2008Edit or Delete MessageReport This Post
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This is all very interesting and makes sense. I was interested in the part of the dry brittle hair. My daughter is the only one in the family to have very dry hair, the rest of use have oily hair and skin.
 
Posts: 142 | Location: PA | Registered: June 23, 2006Edit or Delete MessageReport This Post
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Note, there are other things that can cause dry, brittle hair. Usually a deficiency though. What the deficiency is/ type of deficiency could be different for each person.

But here's some things I put together with hair............

"The tropocollagen or "collagen molecule" subunit is a rod about 300 nm long and 1.5 nm in diameter, made up of three polypeptide strands, each of which is a left-handed helix, not to be confused with the commonly occurring alpha helix, which is right-handed. These three left-handed helices are twisted together into a right-handed coiled coil, a triple helix or "super helix", a cooperative quaternary structure stabilized by numerous hydrogen bonds. Tropocollagen subunits spontaneously self-assemble, with regularly staggered ends, into even larger arrays in the extracellular spaces of tissues. There is some covalent crosslinking within the triple helices, and a variable amount of covalent crosslinking between tropocollagen helices, to form the different types of collagen found in different mature tissues — similar to the situation found with the α-keratins in hair."

"α-keratins [are found] in the hair (including wool), horns, nails, claws and hooves of mammals."

"Keratins contain a high proportion of the smallest of the 20 amino acids, glycine, whose "side group" is a single hydrogen atom; also the next smallest, alanine, with a small and noncharged methyl group"

"In addition to intra- and intermolecular hydrogen bonds, keratins have large amounts of the sulfur-containing amino acid cysteine, required for the disulfide bridges that confer additional strength and rigidity by permanent, thermally-stable crosslinking—a role sulfur bridges also play in vulcanized rubber. Human hair is approximately 14% cysteine."


If there's a block at Homocysteine due to a malfunctioning Methylation Cycle, then this builds up and there is not enough Cysteine and Glycine for later on down the cycle.
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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"Epidimology:
Commonest cause of homocystinuria in patients of Celtic origin. Guthrie testing has shown the incidence to be 1 in 157,000, but is much higher in Ireland (1 in 65,000). All cases are inherited as autosomal recessive. 50% are responsive to pyridoxine (vitamin B6) and tend to have milder disease.1"

http://www.patient.co.uk/showdoc/40002432/

Too bad there's not a test for Austria! The now stated beginning homeland of the Celts is Austria.
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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Nitekitty - I am designating you the official rocket scientist of the group! Pretty impressive that you take the time to put together all this research - thanks for sharing it. I found it interesting because not only am I double jointed but I developed severe allergic reactions to sulphur drugs right about the time I was diagnosed with POTS. Interesting stuff....I will ask my Dr. next time I see him about checking the homocysteine levels....
 
Posts: 570 | Location: Delaware | Registered: February 03, 2008Edit or Delete MessageReport This Post
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Interesting stuff!

As a member of the healthcare system, I would like to say, we are not all bad! It's like anything else, there is good and bad everywhere. Having said that, remember that doctors treat diseases not people. Nurses should be advocating for their patients. I'm glad you are all taking the initiative to get answers; I know first hand that it can be very frustrating.

Wink
 
Posts: 95 | Location: CT | Registered: April 09, 2008Edit or Delete MessageReport This Post
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Sushila and I research and swap articles and notes. So she definitely gets some credit for the stuff I posted above! Wink Smiler
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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I posted this lowered Serine levels thing on its own blog, but since it has to do with the theory I posted up above, I'm re-posting it here as well.

http://www.ncbi.nlm.nih.gov/pubmed/3100911?ordinalpos=2...anel.Pubmed_RVDocSum

Homocysteinemia: depressed plasma serine levels.

Dudman NP, Tyrrell PA, Wilcken DE.

Plasma serine levels were found to be lower than normal (mean +/- SD, 91 +/- 18 mumol/L, n = 16) in homocystinuria patients with a deficiency of cystathionine B-synthase on folate therapy, compared with healthy adults (121 +/- 25 mumol/L, n = 25, P less than 0.001). Of 13 other patients with elevated plasma total homocysteine, two patients with homocystinuria due to remethylation defects had normal serine levels, while 11 renal transplant recipients with mild elevations of serum creatinine had lower than normal serine levels (100 +/- 28 mumol/L, P less than .05). Treatment of both the pyridoxine responsive and nonresponsive cystathionine B-synthase-deficient patients with betaine, which lowered plasma homocysteine, also normalized plasma serine levels. In the two patients with remethylating defects however, betaine lowered plasma homocysteine levels without changing plasma serine levels. By contrast, treatment of the renal transplant patients with pyridoxine, folic acid, and vitamin B12 (cofactors required for homocysteine metabolism), caused falls in plasma homocysteine levels, with a concurrent decline in plasma serine levels. These findings may be explained in terms of the requirements for serine in homocysteine metabolism, both as a source of methyl carbon atoms in the methylation of homocysteine by N5-methyltetrahydrofolate and as a substrate in the cystathionine B-synthase reaction. During periods of elevated plasma total homocysteine in man, increased amounts of serine may be diverted to lowering plasma homocysteine.

PMID: 3100911 [PubMed - indexed for MEDLINE]
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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If I can draw it all out on some paper, there's a sort of tri-fecta if you will:
Methylation Cycle, Renal System, Hypothalamus.
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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Since a bunch of people had asked about it..... As far as brittle hair--- looks like glycine is okay for me, however, serine is on the low end and Cysteine was not tested at all. "Human hair is approximately 14% cysteine." My homocysteine was sky high.

Apparently Homocystinuria alone can cause brittle hair.
 
Posts: 1491 | Location: Los Angeles, CA | Registered: February 12, 2007Edit or Delete MessageReport This Post
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