Methylsuccinate

ChemistryAlso known as: Methylsuccinic acidAlso known as: 2-Methylsuccinic acidumol/L

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

Normal Range
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umol/L
Abnormal Levels
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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.

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

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