Isobutyrylglycine

Optimal Result: 0 - 3 mmol/mol creatinine.

Isobutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism.

The analysis of acylglycines is important for the diagnosis of inherited disorders of mitochondrial fatty acid beta-oxidation. Quantitative analysis of urinary acylglycine has been used to detect inborn errors of metabolism. 

In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation.

Acyl glycines are produced through the action of glycine N-acyltransferase. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency.

Isobutyryl-CoA dehydrogenase deficiency (IBD deficiency) is an inborn error of valine (an amino acid) metabolism. The symptoms, which may not develop until later in infancy or childhood, can include failure to thrive, dilated cardiomyopathy, seizures, and anemia. IBD deficiency is caused by genetic changes in the ACAD8 gene. It is inherited in an autosomal recessive manner.

Moreover, Isobutyrylglycine is found to be associated with ethylmalonic encephalopathy and propionic acidemia, which are also inborn errors of metabolism.

Ethylmalonic encephalopathy is an inherited disorder that affects several body systems, particularly the nervous system. Neurological signs and symptoms include delayed development and the loss of previously acquired skills (developmental regression), weak muscle tone (hypotonia), seizures, and abnormal movements.

Propionic acidemia is an inherited disorder in which the body is unable to process certain parts of proteins and lipids (fats) properly. It is classified as an organic acid disorder, which is a condition that leads to an abnormal buildup of particular acids known as organic acids.

Note: Isobutyrylglycine is also a biomarker for the consumption of cheese.

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

Acyl glycines are produced through the action of glycine N-acyltransferase. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency.

Isobutyryl-CoA dehydrogenase deficiency (IBD deficiency) is an inborn error of valine (an amino acid) metabolism. The symptoms, which may not develop until later in infancy or childhood, can include failure to thrive, dilated cardiomyopathy, seizures, and anemia. IBD deficiency is caused by genetic changes in the ACAD8 gene. It is inherited in an autosomal recessive manner.

Moreover, Isobutyrylglycine is found to be associated with ethylmalonic encephalopathy and propionic acidemia, which are also inborn errors of metabolism.

Ethylmalonic encephalopathy is an inherited disorder that affects several body systems, particularly the nervous system. Neurological signs and symptoms include delayed development and the loss of previously acquired skills (developmental regression), weak muscle tone (hypotonia), seizures, and abnormal movements.

Propionic acidemia is an inherited disorder in which the body is unable to process certain parts of proteins and lipids (fats) properly. It is classified as an organic acid disorder, which is a condition that leads to an abnormal buildup of particular acids known as organic acids.

10 000+ happy customers
100% satisfaction
★ ★ ★ ★ ★ customer support

Understand blood, stool, and urine medical results from all your laboratories.

Upload Your Personal Results

Upload Personal Results

For professional account plans go here

for professional account plans go here

Monthly plan

Annual plan

Own it for life

  • Subscription
  • Data entry service included

    Data entry service

    Our exclusive data entry service is a convenient way to get your results into your private dashboard. Simply attach an image or a file of your lab test results, and one of our qualified data entry team members will add the results for you. We support all sorts of files, whether PDFs, JPGs, or Excel. This service is excellent whether you have a lot of reports to upload or are too busy to do the data entry yourself.

  • Instant interpretation
  • Use free data entry form

    Free data entry form

    We strive to make the data entry process easy for you. Whether by offering dozens of templates to choose from that pre-populate the most popular laboratory panels or by giving you instant feedback on the entered values. Our data entry forms are an easy, fast, and convenient way to enter the reports yourself. There is no limit on how many lab reports you can upload.

  • 4000+ biomarkers explained
  • Nutrition recommendations
  • Cancel anytime

Complete

$15 /month

billed every month

  • monthly
  • $15 per report
  • ✓ Instant interpretations
  • ✓ 4000+ biomarkers explained
  • ✓ Nutrition recommendations
  • ✓ Free data entry form
  • ✓ Unlimited data
  • ✓ $15 data entry service
  • ✓ Cancel anytime

Most popular

Data entry included

Advanced

$79 /year

$6.60/month billed annually

  • yearly
  • 5 reports free
  • ✓ Instant interpretations
  • ✓ 4000+ biomarkers explained
  • ✓ Nutrition recommendations
  • ✓ We enter 5 reports for you
  • ✓ Yearly subscription
  • ✓ Cancel anytime

Data entry included

Unlimited

$250 /once

own it for life

  • pay once
  • 10 reports free
  • ✓ Instant interpretations
  • ✓ 4000+ biomarkers explained
  • ✓ Nutrition recommendations
  • ✓ We enter 10 reports for you
  • ✓ No subscription
  • ✓ Dedicated support
  • ✓ Unlimited access

Are You a Health Professional?

Get started with our professional plan

Welcome to Healthmatters Pro.

Save time on interpreting lab results with the largest database of biomarkers online. In-depth research on any test at your fingertips, all stored and tracked in one place. Learn more

Pro Monthly Plus

for health professionals

  • ✓ Track multiple clients results
  • ✓ No setup fees
  • ✓ No hidden costs
  • ✓ Instant interpretations
  • ✓ 4000+ biomarkers explained
  • ✓ Includes data entry service
  • ✓ Personal training and support
  • ✓ Cancel anytime

$75 per month

Use promo code to save 10% off any plan.


We implement proven measures to keep your data safe.

At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put industry-leading security standards in place to help protect against the loss, misuse, or alteration of the information under our control. We use procedural, physical, and electronic security methods designed to prevent unauthorized people from getting access to this information. Our internal code of conduct adds additional privacy protection. All data is backed up multiple times a day and encrypted using SSL certificates. See our Privacy Policy for more details.