Methylsuccinate

ChemistryAlso known as: Methylsuccinic acidAlso known as: 2-Methylsuccinic acidmmol/mol creatinine

Measures methylsuccinate, an organic acid, in blood to assess cellular metabolism.

Normal Range
0 – 3
mmol/mol creatinine
Abnormal Levels
< 0 mmol/mol creatinine
> 3 mmol/mol creatinine
Specimen Type
Blood

Why This Biomarker Matters

Elevated levels indicate inherited metabolic disorders; early detection enables dietary and medical management to prevent neurological and organ damage.

Optimal Ranges

Clinician-defined 4-point reference thresholds (mmol/mol creatinine)

Default Range

0
0
3
3
Low
0 mmol/mol creatinine
Borderline Low
0 mmol/mol creatinine
Optimal
3 mmol/mol creatinine
Borderline High
3 mmol/mol creatinine

Overview

Methylsuccinate is an organic acid produced during cellular metabolism. Elevated blood methylsuccinate levels are associated with inherited metabolic disorders affecting how your body breaks down certain amino acids and fats, particularly methylmalonic acidemia and related conditions. These rare genetic disorders can cause neurological problems, developmental delay, and organ damage if unmanaged. This test is part of expanded newborn screening panels and is used in metabolic disease evaluation. Identification allows for early dietary management and supplementation to prevent serious complications.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

69829-0Primary
Methylsuccinate [Moles/volume] in Serum or Plasma
umol/L
30479-0
Methylsuccinate [Presence] in Urine
69828-2
Methylsuccinate [Moles/volume] in Amniotic fluid
umol/L
82503-4
Methylsuccinate [Moles/volume] in DBS
nmol/mL

Available Lab Tests

Order tests that measure this biomarker

Urinary Organic Acids (94 biomarkers)

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