Isobutyrylglycinuria (IBG)


Isobutyrylglycinuria (IBG) is an inborn error of metabolism, an inherited condition in which an enzyme in the body, isobutyryl-CoA dehydrogenase, is either missing or not working well. The job of this enzyme is to help break down the amino acid valine (a part of protein). Isobutyrylglycinuria is an organic acid condition because it can lead to the build-up of harmful amounts of organic acids and toxins in the body. Support groups are available to help connect families living with isobutyrylglycinuria with others who have experience or expertise with this condition.

To learn more about Isobutyrylglycinuria, visit Baby's First Test: Isobutyrylglycinuria and Screening, Technology, and Research in Genetics (STAR-G): Isobutyryl-CoA dehydrogenase deficiency. Please see Medically Prescribed Formula for information about medically prescribed formula and pharmacologic interventions for inborn errors of metabolism. A listing of contact information for Minnesota inborn errors of metabolism medical specialty providers/clinics serving children affected with or undergoing evaluation for conditions that can be detected through Minnesota Newborn Screening is available upon request.