Saturday, January 17, 2015

GSD IIIa, ketones, MAD and Veech again

I mentioned the high protein/exogenous ketone approach to Glycogen Storage Disease IIIa in a recent post. It's very nice that an effective treatment can actually be achieved through a Modified Atkins Diet (MAD at <10g of carbs per day) involving food, w/o faking ketosis though those exogenous synthetic ketones.

Robert gave me the heads up on the latest paper on GSD IIIa using MAD, available as a free text through Pubmed. It's rather good as, again, it shows that there are medics out there who think matters through and occasionally come to correct conclusions. I love the clinical details of compliance/non compliance too. And that the early hypoglycaemia, treated with corn starch (bleugh), was asymptomatic under ketosis.

That's nice.

He also sent me the full text of Veech's

The Determination of the Redox States and Phosphorylation Potential in Living Tissues and Their Relationship to Metabolic Control of Disease Phenotypes

which is a fascinating personal insight in to what it was like to work in Hans Krebs' lab, combined with the sort of hard core math which implies rather more understanding of biochemistry than simply adding MitoSOX red to some cells and looking for colour changes to show oxidative stress. That's some complex reading to work through when clinical and home life combine to give me a chance!



ItsTheWooo said...

I think my comment got eaten :(

Anyway great entries Peter and nice to see you blogging again.

Anecdote re: hypoglycemia in ketosis, a few days ago I experimented with lowering protein intake, with expected result of higher ketones from less glucose. My sugar often runs low I'm keto without symptoms,b but two days ago I noted particularly purple pee and was curious what my sugar was, and discovered it was 48. I checked my blood ketones at that time and they were only 1.3.

In spite of blood sugar of 48 and ketones only of 1.3, I had no symptoms at all of neuroglycopenia.

I consider this evidence of following
1.protein is an important source of blood sugar
2.keto adaption involves significant direct use of ffa for energy both body and brain, as low blood sugar and low ketones peripheral dis not produce symptomatic hypoglycemia. My brain ketones were likely higher from ffa transport being increased, whereas my body was oxidizing ffa directly for energy...thus, no sx in spite very low blood level of nutrients.

Interesting and thought I would share

Peter said...

Hi Wooo, that's cool. I spent so long on the hyperglycaemia paper trying to see the logic to down regulating pancreatic glucokinase in fasting i.e. metabolism is quite happy for a day or two of hypoinsulinaemic hyperglycaemia when mixed food becomes available at the end of a period of extended fasting. But hyperglycaemia combined with hyperinsulinaemia appears verboten physiologically and bad in the paper. But the paper never quite lets you see in to this clearly.......... BTW I'm not very good at commenting on the net but Scribble Pad is compulsory reading


Passthecream said...

Hi Peter,

thanks yet again. I stumbled across this ketone ester paper with input by Veech, free full text available:

did you already comment on this one? Freeze-blowing sounds like an unpleasant end! Rats->zombies.


Spittin'chips said...

G'day Peter, was surfing the Hyperlipidnet recently - around about the time I discovered Easiyo yoghurt makers. Your post from 2008 - do you still use them? If so, any tips to pass on?

wellnesswish said...

why not just buy creme fraiche in shop? fermented cream and nothing else?

Team Kruse said...

Peter the redox potential is going to be the next key metric in medicine. University of Virginia heart surgeons are now using it as the main indicators of for and against surgery and post operative therapies. The issue is how best to measure it clinically. Right now we have non specific ways to do it clinically and this is how I use labs to draw inferences to the intracellular state. It determine how biochemistry unfolds in us.

gallier2 said...


I discovered recently an entry in one of my microprocessor blogs about cell biology. It was quite surprizing, because the blogger has nothing to do with metabolism and health stuff, he's a real hardware engineer of exceptional knowledge.
He doesn't pretend to know anything about cells, but he was fascinated by his misconceptions in face of articles he read.
He presents several animations and factoids who are really surprizing when viewed from an engineers point of view (the proton pump being in fact an electric motor with a speed of 40000 rpm and such).
Interesting read.

George Henderson said...

@Spittin' Chips,
I've tried the EasyYo yughurt maker with customised pharmaceutical grade probiotics as culture, and real milk.
Some interesting results.

Spittin'chips said...

@George - I'll take a stab and assume it was good-interesting rather than funky/nasty-interesting.

@wellnesswish - if you were responding to me - creme fraiche isn't common where I live and works out to be a bit more than 5 times the cost of home-made yoghurt.


Passthecream said...

@Spittin' Chips,

I get good results with one of the simple 1 litre $30 makers you can find on ebay. I take 800ml whole milk (Paris Creek unhomogenised, at a shop near you in SA) and add 200 ml of pure cream and brew it overnight.

btw I was amused by your experiences with bamboo, I have a similar photo of a trailer full of arundo donax roots.


Spittin'chips said...

Thanks, Passthecream.

Have a good one.

Silver said...

Peter, Dr. Bernstein states in his internet talk, from December 14, 2014, here is the link,

in answer to a question from a Type 1 diabetic: "Could eating butter raise blood sugar, due to fatty acids in the blood causing the liver to convert protein to sugar? (at 29:55 on the MP3 audio recording),

Dr. Bernstein states that when there are high levels of FFAs in the blood, that the liver sees, that eating butter might cause the blood sugar to increase, the liver gets permission to convert amino acids to glucose, and, also the blood sugar could rise due to the incretin effect ("the Chinese Restaurant Effect"), when GLP1 (Glucagon-like-Peptide) tells the pancreas to make insulin and glucagon. If the diabetic cannot make insulin, only glucagon will be produced.

Dr. Bernstein said on the recording, that this question was the most intelligent question he'd seen in a long time.

Silver said...

Oops, Dr. Bernstein's talk was on December 30th.

He also said that he explains about the amino acids being converted to amino acids in the presence of high FFAs in his talks on youtube.

Silver said...

I'm tired, sorry for the typing errors.

That's "amino acids into glucose"

George Henderson said...

@Spittin' chips,
nice flavour/texture but most interesting how digestibility and tolerability of milk was improved.

Silver said...

George, I started taking all my cream and butter in cultured/fermented form, avoiding sweet cream and butter, and hard cheeses. As a result of this, many of my food allergy/intolerance symptoms disappeared.

I use Homestead Creamery half-'n'-half and their heavy whipping cream, which are pasteurized, not ultrapast., and not homogenized. That's the closest to raw cream I can get where I live.

Peter posted some years ago about several health troubles clearing up, when taking all his cream in fermented/cultured form. That inspired me. :)

Took me a while to give up the sweet cream and butter, etc., but it was worth it.

Peter said...

Hi all,

I made fermented cream in the Easy Yo for about four years and in the end time pressure, mostly, retired it. I liked the ability to adjust the acidity obtained (mostly the more the better). Down side was the occasional contaminant culture. But with the hours I work nowadays just keeping the chickens looked after doesn’t leave much to spare!

My children still drink fresh milk and the bugs in non pasteurised milk from grass fed Jersey cows seems to suit them quite well. I drive past this place every day, except when I stop to buy milk of course:

I've left a litre at room temp to ferment in its own bugs in the past and it makes a nice buttermilk type drink, if a little high in protein for my preferences, so not often.


Spittin'chips said...

It seems you are lucky to have access to such a well-run dairy. Our laws in the penal colony are heading further in the opposite direction.

A toddler died late last year from haemolytic uraemic syndrome, apparently. The coronial inquest hasn't been finalised, but the pollies are not known for waiting until all facts are known. That would be too logical.

Passthecream said...

I had a neighbour who supplied fresh goat milk to most of the Adelaide metro area. The powers-that-be made him print "Warning Contains Raw Unpasteurised Goat Milk" on the containers in letters so large that you couldn't read the rest of the label. Obviously it is much more dangerous than smoking.


raphi said...

"The Determination of the Redox States and Phosphorylation Potential in Living Tissues and Their Relationship to Metabolic Control of Disease Phenotypes"

was one of the most entertaining 'science' reads as of late.