A healthy result should fall into the range 0 - 0.04 µg/g creatinine.

Tiglylglycine (TG) is associated with both mitochondrial and/or genetic disorders. Toxic chemical exposure may be one of the most common causes of mitochondrial dysfunction. In mitochondrial disorders of the respiratory chain, TG values are usually more moderately increased than in the genetic disorders. In the medical literature, a normal value is less than 3.8 mmol/mol creatinine in children.

Tiglylglycine result calculator

insert the value from you Tiglylglycine test result.

What does it mean if your Tiglylglycine result is too high?

It is an intermediate product of the catabolism of isoleucine and ketone bodies. TG is found at variable high concentration in the urine of patients with 2-methylacetoacetyl-CoA thiolase or 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiencies, which are inherited neurometabolic disorders affecting isoleucine catabolism. Biochemically, 2-methylacetoacetyl-CoA thiolase deficiency is characterized by intermittent ketoacidosis and urinary excretion of 2-methyl-acetoacetate (MAA), 2-methyl-3-hydroxybutyrate (MHB) and tiglyglycine (TG), whereas in MHBD deficiency only MHB and tiglylglycine accumulate. Typical clinical symptoms in both disorders include intermittent ketoacidotic episodes, seizures, and retardation. These diseases can be treated by switching to a diet low in protein and without isoleucine. In some cases, patients were asymptomatic until provoked by vaccinations or viral infection. In both disorders, biochemical abnormalities became more pronounced after a 100mg/kg oral isoleucine challenge. Tiglylglycine is also moderately elevated in short-chain acyl dehydrogenase (SCAD) deficiency, in propionyl CoA carboxylase, in methylmalonic aciduria, in the mitochondrial disorder Pearson syndrome (caused by mitochondrial DNA deletion), and in disorders of the respiratory chain in mitochondria.

Abnormal results may be confirmed by advanced mitochondrial DNA testing. Normal values of lactate and pyruvate do not rule out the presence of mitochondrial disorders; elevated TG should be considered a better marker of mitochondrial dysfunction than lactate or pyruvate levels. Extremely elevated values are likely due to genetic chromosomal mutations. Confirmation of genetic disorders requires DNA and/or enzyme testing at advanced biochemical genetics centers. Coenzyme Q-10 (300-600 mg), NAD 25 mg, L-carnitine and acetyl-L-carnitine (1000-2000 mg), riboflavin (40-80 mg), nicotinamide (40-80 mg), biotin (4-8 mg), and vitamin E (200-400 IU’s) per day may improve mitochondrial dysfunction. Hyperbaric oxygen therapy (HBOT) may also be beneficial. Discuss specific treatment regimen with your medical doctor.

Are you concerned about your lab report?
Select your plan, upload your lab reports and we’ll provide you with a meaningful interpretation.

Basic Interpretation
  • Instant interpretation
  • Use self upload form
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Cancel anytime
Full Interpretation
  • Instant interpretation
  • We upload 4 reports for you
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Cancel anytime
  • Online support
Own It For Life
  • Unlimited access
  • No subscription
  • Instant interpretations
  • We upload 10 reports for you
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Dedicated support

Test results don't lie. Learn what is happening inside your body.

Health is not exactly rocket science. You just have to know what your body needs and doesn't need. Your medical tests contain that information. Upload them to our service and we’ll help you understand, organize, and act on them.

Get Started

Medical results explained and interpreted.


Healthmatters.io customer review

"I was really concerned about my lab results and my GP was not able to see me for another week. Through HealthMatters I was able to interpret my results instantly."

Healthmatters.io thankfull female customer

"I've been keeping all my lab results in a spreadsheet, covering approximately 20 years. The HealthMatters team helped me upload that info into the cloud. I can show this data to my family and doctors now easily."

Healthmatters.io thankful young female customer

"I had no idea what my lab results meant. Here I found all the biomarkers that I was looking for online. Finally, I have not only organized them all neatly in one place, but I am also grateful to be able to understand what they mean."

Privacy and security of personal data. HIPAA and GDPR compliant badges.

Your Privacy And Security

At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put security measures in place to help protect against the loss, misuse or alteration of information under our control. We use procedural, physical and electronic security methods designed to prevent people who aren't authorized from getting access to this information. Our internal code of conduct adds additional privacy protection. See our Privacy Policy for more information.