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(Glycogenosis) The many roads to Glycogen Storage Depletion

Discussion in 'General ME/CFS Discussion' started by Radio, Jan 10, 2014.

  1. Radio

    Radio *****

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    I started this thread to show there are many roads to Glycogen Storage Depletion

    Glycogen Storage Diseases (Glycogenosis)
    Glucose derived from glycogen breakdown is an important source of energy for the body. It provides energy for muscle contraction, and serves as the primary source of energy for the brain. Healthy people maintain a relatively constant 5 mM blood glucose concentration to support tissues and organs.
    http://www.uic.edu/classes/phar/phar332/Clinical_Cases/carbo metab cases/glycogen metab/Glycogen metabolism.htm

    Glycogen Breakdown
    http://oregonstate.edu/instruct/bb450/summer09/lecture/glycogennotes.html
    10 Steps of Glycolysis
    http://biology.about.com/od/cellularprocesses/a/aa082704a.htm

    Debranching enzyme
    is a molecule that helps facilitate the breakdown of glycogen, which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. Together with phosphorylases, debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels.[1] When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result.
    http://oregonstate.edu/instruct/bb450/summer09/lecture/glycogennotes.html

    Gluconeogenesis
    http://en.wikipedia.org/wiki/Gluconeogenesis
    Kynurenine Pathway
    Viruses and mineral depletion can affect the NAD Synthesis in the Kynurenine pathway. We know quinolinic is elevated in CFS/ME subgroups.The main concern is the NADH recycling anaerobic metabolism (ADP to ATP) imbalance. If we have a metabolic imbalance of NAD+ NADH, this can impact the metabolism of glycogen and possibly cause Glycogenosis storage depletion.
    • Gluconeogenesis begins in the mitochondria with the formation of oxaloacetate by the carboxylation of pyruvate. This reaction also requires one molecule of ATP, and is catalyzed by pyruvate carboxylase. This enzyme is stimulated by high levels of acetyl-CoA (produced in β-oxidation in the liver) and inhibited by high levels of ADP.
    • Oxaloacetate is reduced to malate using NADH, a step required for its transportation out of the mitochondria.
    http://en.wikipedia.org/wiki/Kynurenine_pathway
    Glycogen Storage Metabolic Syndrome?
    There is a consistently high Methylhistidine on OAT testing in the chronic fatigue community, could this contribute to a glycogen storage problem?


    Chronic-Infection-H-Pylori-Manganese-Connection?
    http://forums.phoenixrising.me/inde...nfection-h-pylori-manganese-connection.27569/

    Last edited: Jan 17, 2014
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  2. alex3619

    alex3619 Senior Member

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    Now you are back to where I was in 1997. My first model made a link between Tarui Disease (glycogen storage disease VII) and CFS. For a while I was called the phosphofructokinase guy. I postulated that phosphofructokinase was suppress in CFS by high concentrations of citric acid, and further postulated this might be due to a block at the Krebs Cycle enzyme aconitase. This disorder is accompanied by high uric acid. Several lines of research confirmed this until it was shown we have high phosphofructokinase activity.
  3. xchocoholic

    xchocoholic Senior Member

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    Tx.I was looking at thus at one point.

    I realized several years agothat I need increase my blood glucose every 20 minutes if I'm active. More often if I'm doing any aerobic activity like cleaning.

    McArdles is interesting in that patients have burgundy colored urine post exercise. I wonder if there's a kidney stone or oxalate connection.
    Last edited: Jan 11, 2014
  4. Radio

    Radio *****

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    Hey Alex,
    My research leads me to the conclusion that the mitochondria damage contributes to the Glycogen Storage problem. The methylation dysfunction and mineral deficiencies can causes metabolic waste to builds up and over-loads the system. I also wanted to make a suggestion that eating a super low carb diet may not be a good idea, If there is in fact a possible Glycogen Storage factor. Treatment options? (Glucose Insulin Therapy), could be a possible treatment options for CFS. (GIT) could promote more glycogen storage into the cells and help drive the krebs cycle to make more ATP. Also, supplementing NAD+ may improve anaerobic metabolism (ADP to ATP) imbalances.
    Last edited: Jan 17, 2014
  5. lansbergen

    lansbergen Senior Member

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    I have never seen that.

    Urine from Vit B use can be very dark. Are you sure it is not that?
  6. xchocoholic

    xchocoholic Senior Member

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    That was in the first link.
  7. alex3619

    alex3619 Senior Member

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    A glycogen storage disease should be identifiable with a muscle biopsy. You would think that in half a century and maybe thirty or more million patients somebody would have noticed. Yet they missed exercise testing at the anaerobic threshold since at least 1963, and perhaps '49. So what else have they missed? If you don't look for something, sure enough you wont find it except by chance.

    In the 90s the researchers refused to consider the mitochondria, and indeed insisted that we have accelerated glycolysis. I wound up arguing with a couple of researchers over this, though argument might not really describe the small number of emails we exchanged. They refused to consider that a mitochondrial defect could cause metabolites to rise all the way up the glycolytic pathway.
  8. rwac

    rwac Senior Member

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    There are other reasons why you might have problems storing glycogen.

    For instance glucagon triggers (indirectly via GH and ACTH) cortisol release which can trigger release of glycogen stores and cause suppression of glycogenosis.

    If you're generally stressed (hormonally) that will suppress glycogenosis.

    A low carb diet is not conducive to glycogenosis because of constant use of glucocorticoids to manufacture glucose via gluconeogenesis.

    A hypothyroid state can reduce glycogenosis too: http://www.ncbi.nlm.nih.gov/pubmed/3131128
    Radio likes this.
  9. rwac

    rwac Senior Member

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    Might also consider consuming sugar, since fructose + glucose increases stored glycogen more than either alone.
    http://www.ncbi.nlm.nih.gov/pubmed/3023336
    (There's also a human study on this which I can't find at the moment)
    Radio likes this.
  10. Radio

    Radio *****

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    Interesting, Sugar can be problematic and very damaging to the nervous system. It's also a mast-cell activator, so we need to contemplate the benefits of consuming sugar. Unfortunately there is no easy solution to this problem. Thanks for the post...
    Last edited: Jan 12, 2014
  11. rwac

    rwac Senior Member

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    Hmm, do you have links for that?
    I ask, because I suspect it might not be true.

    another link for fructose and glycogen: http://easacademy.org/research-news...e-postexercise-human-liver-glycogen-synthesis
  12. Radio

    Radio *****

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    High blood glucose (Sugar) can causes nerve damage and is link to the complications we see in diabetes. The latest research has confirmed that the earth is round and rotates the sun.
    I also wanted to add that (B12 deficiency), (MTRR, MTR) genetic disorders are affected by high blood sugar as well...Unfortunately there is no easy solution to these problems. I do see your point...But we have to factor in all the other metabolic dysfunctions. We are in fact on the same page. Check it out, We need the extra Insulin to control and drive the Glucose therapy into the cells with out creating more oxidative stress.
    Last edited: Jan 12, 2014
  13. rwac

    rwac Senior Member

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    Well, there's a big difference between high blood glucose and eating sugar. Sugar is not even implicated in causing diabetes.

    I only have one MTR and some MTHFR polymorphisms so I can't say too much about the genetic issues.
  14. xchocoholic

    xchocoholic Senior Member

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    I'm using dextrose, dex4, to increase my blood glucose and it works better than anything else. So, I'm hesitant to throw sugar under the bus too.

    I have chronic LOW blood glucose per hyperinsulinemia. Blood glucose test was normal except insulin was too high. I was told not many doctors look for this so it gets missed.

    As a celiac with chronic digestive problems, I suspect missing enzymes are affecting this.
    Radio likes this.
  15. rwac

    rwac Senior Member

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    I'm curious now, have you tried eating a spoonful of sugar instead?
    Or perhaps orange juice?
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  16. Radio

    Radio *****

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    Well, there's a big difference between high blood glucose and eating sugar. Sugar is not even implicated in causing diabetes.

    Great info guys,
    I never said that high blood glucose is implicated in causing diabetes, But can be problematic and is a key factor in the the complications we see in diabetes. One of the other issues i have with sugar is the chronic H-pylori / Yeast issues that is very common in CFS. H-pylori overgrowth can contribute to the intercellular mineral deficiencies, as well as the B-12 problem. Maybe once these infections are under control, sugar could be beneficial in increase blood glucose and driving this metabolism.
    Last edited: Jan 17, 2014
  17. Radio

    Radio *****

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    Blood glucose test was normal except insulin was too high. This could lead to insulin resistance and muscle wasting and possibly CFS symptoms.
    Last edited: Jan 12, 2014
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  18. xchocoholic

    xchocoholic Senior Member

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    Yes. Dye free dex4 works best for me.
    typically need 2-3.

    The others don't clear up my head as quickly. I have petite mals, most of the time from pots, and these help.
    Last edited: Jan 12, 2014
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  19. xchocoholic

    xchocoholic Senior Member

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    Yeh. I'm pretty much screwed here. lol.

    I'm on a wahls / paleo type diet so I haven't thrown caution to the wind.
    The MAD modified atkins didn't work for me because my blood glucose levels stayed too low for me to function.

    I need sugar to resolve petite mals but ...

    At 58 and 24 years into me/cfs, I'm to the point of enjoying life and not worrying about what might happen down the road.
    Last edited: Jan 12, 2014
    Radio likes this.
  20. xchocoholic

    xchocoholic Senior Member

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    I was googling digestive enzymes, elastase, maltose, etc., and found connections to diabetes.

    I just re-started Enzymedica Digest, need and improved version, and am noticing more energy. I took these several years ago and forgot. Duh !

    My labs showed low elastase twice so my need for digestive enzymes is established.

    This is an interesting avenue seeing as how fatigue and muscle weakness are linked to glucose.
    Radio likes this.

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