Propionylglycine

ChemistryAlso known as: PGN/A

Measures an organic acid in urine reflecting amino acid and fatty acid metabolism.

Normal Range
Consult your provider
N/A
Abnormal Levels
Consult your provider
Specimen Type
Blood

Why This Biomarker Matters

Elevated propionylglycine indicates possible organic acid disorders requiring urgent dietary management. Early detection prevents seizures, developmental problems, and organ damage.

Overview

Propionylglycine is an organic acid found in urine that indicates how your body metabolizes certain amino acids and fatty acids. Elevated levels can suggest propionic acidemia or methylmalonic acidemia, rare inherited disorders that disrupt metabolism and can cause seizures, developmental delays, and organ damage. This test is particularly important in newborn screening programs and for investigating unexplained symptoms in infants and children. Early detection enables specialized dietary management and medical treatment to prevent serious complications.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

32584-5Primary
Propionylglycine [Presence] in Urine
47878-4
Propionylglycine [Moles/volume] in Plasma
umol/L
54332-2
Propionylglycine [Moles/volume] in Cerebral spinal fluid
umol/L

Available Lab Tests

Order tests that measure this biomarker