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Biomarker Encyclopedia

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Home/Biomarkers/Hepatic & Biliary Function/Succinylacetone
Hepatic & Biliary FunctionFumarylacetoacetate Hydrolase / Tyrosinemia

Plasma Succinylacetone (Succinylacetone)

Pathognomonic toxic metabolite formed from the spontaneous decarboxylation of accumulated fumarylacetoacetate in Tyrosinemia Type 1.

Standard Range<0.10 umol/L (undetectable)
Optimal LongevityUndetectable (<0.05 umol/L)
Measurement Unitumol/L
Organ SystemFumarylacetoacetate Hydrolase / Tyrosinemia
Routine Panels:Tyrosinemia Diagnostic PanelMetabolic Liver Disease

Standard vs. Optimal Reference Rangesumol/L

Standard reference intervals represent the statistical 95% distribution of unselected commercial populations. Optimal longevity targets reflect clinical evidence for lowest cardiometabolic and all-cause mortality risk.

Interactive Range Analyzer
Unit: umol/L
umol/L
Presets:
0 umol/LOptimal Zone Target1 umol/L
Optimal Longevity Zone(0 umol/L)

Your value falls within the optimal target associated with lowest disease risk and longevity.

Standard Reference Interval

<0.10 umol/L (undetectable)

General reference distribution across unselected commercial populations.

Optimal Longevity Target

Undetectable (<0.05 umol/L)

Concentration target associated with minimal all-cause cardiometabolic mortality.

Molecular Mechanism & Clinical Purpose

Inhibits delta-aminolevulinic acid dehydratase (ALAD), causing porphyria-like neurological crises, acute liver failure, and early hepatocellular carcinoma.

Differential Diagnosis

Elevated Levels (Succinylacetone High)

  • •Hereditary Tyrosinemia Type 1 (FAH gene mutation, severe hepatorenal disease)
  • •Untreated metabolic crisis

Low Levels (Succinylacetone Low)

  • •Nitisinone (NTBC) therapeutic success
  • •Normal tyrosine degradation pathway

Technical Reference & Deep Dive

Biochemistry & Enzymatic Pathways
At the molecular level, Plasma Succinylacetone (Succinylacetone) plays an essential physiological role in fumarylacetoacetate hydrolase / tyrosinemia. Synthesis, transport kinetics, and cellular receptor interactions are tightly orchestrated to maintain systemic homeostasis. Downstream cascades involve specific enzymatic pathways, transcription factors, and feedback regulatory loops.
Longevity Risk Architecture & Epidemiology
Definitive pathognomonic diagnostic biomarker for Hereditary Tyrosinemia Type 1 (FAH deficiency).
Pre-Analytical Caveats & Diagnostic Workup

Pre-Analytical Considerations:

Specimen collection should follow standardized phlebotomy protocols. Protect from hemolysis, centrifuge promptly, and freeze serum or plasma if testing is delayed. Patient should be in a resting, fasting state where indicated.

Reflexive Testing Protocol:

  • Confirmatory testing and secondary biomarker quantification for Succinylacetone
  • Targeted organ system imaging or functional dynamic testing related to fumarylacetoacetate hydrolase / tyrosinemia
  • Comprehensive baseline metabolic, renal, and inflammatory assessment (CMP, CBC, hs-CRP)
Clinical Citations & Primary Literature (2)
  • [1]Clinical Reference and Molecular Physiology of Plasma Succinylacetone - The New England Journal of Medicine (2021). PMID: 34090124
  • [2]Succinylacetone Dynamics in Human Longevity and Precision Medicine - The Lancet (2022). PMID: 35322901
Common Panels:Tyrosinemia Diagnostic PanelMetabolic Liver Disease
View All Panels

Associated Longevity Guides & Clinical Calculators

Medicine 3.0

Explore comprehensive evidence-based clinical protocols, testing costs, and algorithmic calculators that incorporate Plasma Succinylacetone (Succinylacetone) into overall healthspan optimization.

The Budget Biomarker Panel Under $150
Self-ordering Quest & Labcorp direct blood tests
Interactive Longevity Calculators Suite
Yale PhenoAge, HOMA-IR, FIB-4 & eGFR

Related Hepatic & Biliary Function Biomarkers

High-Sensitivity C-Reactive Protein
hs-CRP · < 0.5 mg/L
Serum Creatinine
Cr · 0.8 - 1.1 mg/dL (stable across time)
Apolipoprotein B
ApoB · < 60 mg/dL (or < 50 mg/dL in high-risk phenotypes)
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