OXPHOS And Mito Complex Defect

[Highserotonin90]

@Highserotonin90

What was your experience with ketogenic dieting like. When did you get the recommendation, how did you do it, for how long, what got better, and what got worse?

It is always positive in the first part of the day then regardless of whether I am on a diet rich in sugar or fat I can suffer I call them "crashes" where the first symptom is the retina (see the biochemistry of metabolism books where they talk about it) then all the other neurological symptoms ... muscle ... hypotonia ... language ... logic ...

 

[Highserotonin90]

I really thank everyone who is giving me support ... nobody understands me in Italy ... but I would like to try to lead a normal life.

 

[Recoen]

I have no defects in complex 1 and therefore the excess of NADH comes from the B1 which activates the PDK.

You might not have a defect with complex one but where are all those “electrons” supposed to go if you’re flooding complex II with FADH2?

 

[Highserotonin90]

You might not have a defect with complex one but where are all those “electrons” supposed to go if you’re flooding complex II with FADH2?

Where is it?

 

[Highserotonin90]

If there were problems obtaining pyruvate through glycolysis, would it still be more suitable to use fat as a source of energy?

 

[Highserotonin90]

I haven't run the analysis yet but what if it was G6DP? isn't there a cure?

 

[Tristan Loscha]

I still don't know what defects I have because unfortunately I haven't done a genetic test and a biopsy yet.

I can tell you that since I was born I have had significant neurological problems due to ATP deficiency and physical ... at the moment I am making significant progress with vitamin E, Vitamin C, Vitamin A and Vitamin K2 ... unfortunately I am very confused with the dosage and every 2-3 days I experience a general slowdown in progress.


Is there a risk in blood clotting given the continuous use of E, A, K2?


I have episodes of lactic acidosis which are very disabling in addition to the loss of muscle tone, difficulty in gaining weight (muscle), decrease in muscle strength, mental fog ...

I'm much better off without starches but it's hard to be without

I think my Krebs / glycolysis cycle is too accelerated while the mitochondrial transport chain isn't ... does it make sense to inhibit Krebs with NADH in pure form and increase oxidative phosphorylation in the chain?

What do you think of succinic acid?

Thanks

Longer term ketogenic diet or at least low carb 70 - 100g net carbs will subdue the episodes of lactic acidosis, because humans cannot produce meaningful amounts of lactic acid without carbs..

 

[Highserotonin90]

This morning I resumed the Coenzyme A support which contains 500mg calcium pyruvate, 25mg NIacinamide and 25mg of B5.

It was a disaster ... I locked everything again I don't know what to do anymore.

 

[Tristan Loscha]

This morning I resumed the Coenzyme A support which contains 500mg calcium pyruvate, 25mg NIacinamide and 25mg of B5.

It was a disaster ... I locked everything again I don't know what to do anymore.

"I had tried the ketogenic diet but I can't support it because I constantly crave carbohydrates ... at most I can only consume fats during the day but in the evening I eat carbohydrates."

Is that still a correct statement? You do not consume a ketogenic diet because you have a strong appetite for carbohydrates? What do you eat in a day? You overthink a lot of the issues, treatment is empiric because the diseaseprocess is not well understood.

 

[Highserotonin90]

"I had tried the ketogenic diet but I can't support it because I constantly crave carbohydrates ... at most I can only consume fats during the day but in the evening I eat carbohydrates."

Is that still a correct statement? You do not consume a ketogenic diet because you have a strong appetite for carbohydrates? What do you eat in a day? You overthink a lot of the issues, treatment is empiric because the diseaseprocess is not well understood.

In the morning I am terribly hungry ... I should eat at least 50g of fat to satiate myself and start getting started. Does it seem normal to you?

 

[Tristan Loscha]

In the morning I am terribly hungry ... I should eat at least 50g of fat to satiate myself and start getting started. Does it seem normal to you?

yeah, sure, but you should reduce the carbohydrates though.

 

[Highserotonin90]

I can't have a moment of peace I'm trying again the TPP version B1 but it always seems to make matters worse.

Only the HCL version is effective.

The last step will be to try intramuscular injections of B1 HCL for greater and safer absorption.

The oral version of Metabolics is very effective but it would take a lot of ML to reach an effective 50 / 100mg.

The injections are 50/100 mg / ml.

ALA It works intermittently, sometimes it manages to make me use glucose while other times it just creates problems.

I am desperate, if there is someone who wants to help me in private I am available.

The level of muscle and brain ataxia is at a very high level.

A very low-fat diet also helps but is unsustainable. High sugars.

 

[Highserotonin90]

@haidut Do you think your pyruvate-containing product can help me?

 

[Recoen]

I can't have a moment of peace I'm trying again the TPP version B1 but it always seems to make matters worse.

Only the HCL version is effective.

The last step will be to try intramuscular injections of B1 HCL for greater and safer absorption.

The oral version of Metabolics is very effective but it would take a lot of ML to reach an effective 50 / 100mg.

The injections are 50/100 mg / ml.

ALA It works intermittently, sometimes it manages to make me use glucose while other times it just creates problems.

I am desperate, if there is someone who wants to help me in private I am available.

The level of muscle and brain ataxia is at a very high level.

A very low-fat diet also helps but is unsustainable. High sugars.

Have you tried B1 topically?

 

[Highserotonin90]

Have you tried B1 topically?

How should i do ? the fat soluble vitamins I have found work well in the skin but vitamin B1 is water soluble.

 

[Highserotonin90]

I forgot I tried Niacinamide with a maximum dosage of 100mg.

I must say that it actually helps me to use glucose and not fat ... but within 3 days it caused me an increase in water retention and muscle hypotonia.

Should I probably use it with a low fat content? risk of worsening insulin resistance?

 

[Highserotonin90]

Why don't I tolerate the TOP version of thiamine (therefore the non-phosphorylated version) and only tolerate the final HCL version?

 

[Highserotonin90]

TPP sorry

 

[Recoen]

Why don't I tolerate the TOP version of thiamine (therefore the non-phosphorylated version) and only tolerate the final HCL version?

What absorption pathway and what dose did you try?

 

[Highserotonin90]

What absorption pathway and what dose did you try?

@Recoen Oral drops 400mcg and try three time in same days is water solubil vitamin...why you think topical absorb is better ?