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March20 podcast Dr Hazen
anti-TMA pill in a year or 2 ? (scroll 12 mins)

Additional info:
MEBO Karen
at UK Findacure conf 2020

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MEBO Map Testing & Meetups

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MEBO - UBIOME study 2018



MEBO Gut Microbiome Study
"Microbial Basis of Systemic Malodor and PATM Conditions (PATM)"
Funded by uBiome Research Grant

"Microbial Basis of Systemic Malodor and PATM Conditions (PATM)"

Dynamics of the Gut Microbiota in
Idiopathic Malodor Production

Started May 2018 - Ongoing

Current people sent kits : 100/100
3 kits per person


Participation info : LINK English

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BO Sufferers Podcasts



TMAU Petition world
TMAU UK end total:262
TMAU UK ends 23/01/20
TMAU Petition USA end total 204
USA : Moveon open
TMAU (Dominican)
Metabolomic Profiling Study

Start : Aug 2016
Stage 1 : 27 Canadian volunteers to test
Latest click here (26 oct) :
17 samples returned

Note : Stage 1 is Canada only.
Return cut-off date : passed
Analysis can take 6/8 weeks
Analysis start in/before Nov
MEBO Research is a
NORD Member Organization
See RareConnect TMAU

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MEBO Metabolic Malodor Survey (international) for Dr Hazen click here
click to Read more/less

survey for ANYONE who identifies with METABOLIC MALODOR

begun : Oct20
end : no ending for now

Regular readers will know that Dr Stan Hazen et al at Cleveland Clinic are developing a TMA-blocker pill, as they proposed in a 2011 paper that TMAO is a factor in CVD. Recently Dr Hazen and colleagues contacted MEBO as they have always thought they could also help with TMAU. This survey is to give them an idea of the 'state of the community'. It is a "version 1". They may not even look (though they have access permission), but it could be useful to give them an overview of the community

MEBO had a zoom call with Dr Hazen and his team in October. Another zoom call is planned when they have time

This is a GOOGLE FORMS survey

short url for survey :

current participants : 113 (update 18dec20)

Saturday, July 25, 2009

Dimethylglycine dehydrogenase enzyme (DMGDH)

dmgdhd is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase.

Pathway: choline metabolism
Trimethylaminuria is the only real 'accepted' form of metabolic body odor by those few in the medical system who are aware of any form, but another often forgotten about is dimethylglycinuria, to do with saturation of the Dimethylglycine dehydrogenase enzyme. However there seems to be only one recorded case of DMGU, and it's unclear if this is because no-one tests for it or it is so rare.
(2001 dimethylglycinuria case)

There are a lot of similarities with 'official' TMAU, in that it is thought to give off a fish smell. Also the enzyme is part of the choline metabolism. That said, presumably this enzyme deals only with one 'substrate' (it oxidizes dimethylglycine), whereas FMO3 oxidizes 1,000s of substrates (including trimethylamine). The sufferer was also said to have a high creatine kinase blood level, which may be a clue as to this problem (or maybe not). Whilst FMO3 uses riboflavin as a co-factor, DMGDH uses folate as it's co-factor.

It would be interesting to know if they discovered DMGu by using an open-ended look for volatile organic compounds in a mass spectrometer/gas chromatography test, or if they were only looking for DMG. The former would be the best starting test for defining a metabolic body odor problem, since in most cases we don't know what we are looking for yet.

Why only one case has been recorded is not known, however it would seem at the moment that an assumption could be made that DMGu is very rare and that one or more other forms of metabolic body odor are far more common.

Dimethylglycine dehydrogenase is a gene that encodes an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum.

...defects in dmgdh are the cause of dmgdh deficiency (dmgdhd) [mim:605850]. dmgdhd is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. biochemically it is characterized by an increase of n,n- dimethylglycine (dmg) in serum and urine.

Biochemically it is characterized by an increase of N,N-dimethylglycine (DMG) in serum and urine.

Pathway: Amine and polyamine degradation; betaine degradation; sarcosine from betaine...

SwissProt Accession No.: Q9UI17 Dimethylglycine dehydrogenase, mitochondrial precursor; (Homo sapiens); 99% similarity over 866 a.a.

(Last modified July 7, 2009. Version 80.)


Jeanne said...

Are there any clinical trial for DMGDH? I understand there may be a pill coming out to help with TMAU, would that also work for DMGDH? I have the DMGDH gene, it has been 22 years of constant Dr. visits with no help from anyone - I am at the end of my rope, hope is running out. I have no life.

Aug 15, 2016, 11:44:00 AM
Anonymous said...

Are there any clinical trials pending for DMGDH? I understand there may be a pill coming out to help with TMAU, would this also help DMGDH? I have the DMGDH gene and have suffered for 22 years, hope is running out, I have no life.

Aug 15, 2016, 11:46:00 AM
blogcontributor2 said...

Hi. I don't know to be honest. Perhaps the tmau pill will alter the gut flora including microbes that produce DMG. But I really don't know.

I have known someone diagnosed DMGU before (the UK lab often tests tma-negative samples and as far as I know none have been found). I guess you have been diagnosed by the DNA test.

Aug 15, 2016, 12:01:00 PM
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