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Updated: May 19, 2026
Simultaneous Affinity Enrichment of Two Post-Translational Modifications for Quantification and Site Localization
Published on: February 27, 2020
Performance of succinylacetone assays and their associated proficiency testing outcomes
B W Adam1, E M Hall, N K Meredith
1Centers for Disease Control and Prevention (CDC), 4770 Buford Highway NE, Atlanta, GA 30341, USA. BAdam@cdc.gov
Background:
Succinylacetone (SUAC) is the primary metabolic marker for hepatorenal tyrosinemia.
Materials And Methods:
We used results reported for dried-blood-spot proficiency testing (PT) specimens and hepatorenal tyrosinemia patients' newborn screening (NBS) samples to demonstrate analytic biases in SUAC recoveries and differences in presumptive clinical classifications.
Results:
SUAC recoveries from non-kit and NeoBase™ kit tandem mass spectrometry methods were markedly different. Kit users that set high cutoff values submitted discordant clinical assessments of "within normal limits" for PT specimens enriched with 10-15 micromol SUAC/L in blood. SUAC levels in tyrosinemia patients' NBS samples analyzed by NeoBase™ kit were lower than those in samples analyzed by non-kit methods.
Conclusions:
From 2009 to 2011, analytic biases in SUAC recoveries were consistent. Discordant clinical assessments of PT specimens were associated with high cutoff values for NeoBase™ kit results. Method-related differences in SUAC concentrations of tyrosinemia patients' samples were consistent with those of PT specimens.
