Cystathionine

ChemistryAlso known as: Cystathionin 24h Ur QlAlso known as: Cystathionin 24h Ur-sCncAlso known as: Cystathionin 24h Ur-sRatenmol/mL

Measures cystathionine levels in serum or plasma to assess amino acid metabolism.

Normal Range
150 nmol/mL
nmol/mL
Abnormal Levels
Consult your provider
Specimen Type
Blood

Why This Biomarker Matters

Elevated cystathionine suggests a rare metabolic disorder requiring dietary modification and medical management to prevent neurological and physical complications.

Understanding Your Results

Normal Range

150 nmol/mL

Overview

This test measures cystathionine, an amino acid intermediate in the methionine metabolism pathway, in urine. Elevated cystathionine can indicate cystathionine beta-synthase (CBS) deficiency, a rare genetic metabolic disorder that disrupts amino acid processing. This condition can lead to neurological problems, lens dislocation, and cardiovascular complications. Early detection through urine screening allows for dietary management and supplementation to prevent complications.

Research & Evidence

100 publications

Sort:
The Role and Research Progress of Cystathionine β-Synthase in the Female Reproductive System.
Compr Physiol · 2025
PMID: 41159658Review
DPhA-EtOBz-TSC targets cystathionine γ-lyase (CSE) to trigger ferroptosis and inhibit colorectal cancer growth in vitro and in vivo.
Eur J Pharmacol · 2026
PMID: 41621574
Loss of cystathionine-β-synthase contributes to elevated OXPHOS, a vulnerability in Ara-C-resistant Myeloid Leukemia in Down syndrome.
Biochem Pharmacol · 2026
PMID: 41692349
Dietary Organosulfur Compounds and the H₂S-Polysulfide Redox Axis: Current Evidence and Limitations.
J Nutr Biochem · 2026
PMID: 41780765
Corrigendum to "Hydrogen sulfide in boar reproduction: Cystathionine β-synthase and cystathionine γ-lyase localization in reproductive tissues and sperm across maturation stages" [Theriogenology 252 (2026) 117753].
Theriogenology · 2026
PMID: 41565564
Hydrogen sulfide in boar reproduction: Cystathionine β-synthase and cystathionine γ-lyase localization in reproductive tissues and sperm across maturation stages.
Theriogenology · 2026
PMID: 41265009Preclinical
The First NIR Fluorescent Probe for Bioimaging of Cystathionine-γ-lyase Activity in Living Cells and Xenograft Tumor Models.
Anal Chem · 2026
PMID: 41708281
[Retracted] Erastin sensitizes glioblastoma cells to temozolomide by restraining xCT and cystathionine‑γ‑lyase function.
Oncol Rep · 2026
PMID: 41823559
Endothelial cystathionine-γ-lyase (CTH) regulates body weight and insulin resistance in mice fed a high-fat diet.
Redox Biol · 2026
PMID: 41707615
Cystathionine-γ-Lyase/Hydrogen Sulfide Axis Plays a Crucial Role in Promoting the Progression of Cervical Cancer.
J Biochem Mol Toxicol · 2026
PMID: 41797258

Research data from MEDLINE/PubMed · 100 articles

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

2172-5Primary
Cystathionine [Presence] in Serum or Plasma
26964-7
Cystathionine [Moles/volume] in Urine
nmol/mL
25388-0
Cystathionine [Moles/time] in 24 hour Urine
umol/(24.h)
26592-6
Cystathionine [Moles/volume] in Cerebral spinal fluid
umol/L
26607-2
Cystathionine [Moles/volume] in Serum or Plasma
umol/L
53066-7
Cystathionine [Moles/volume] in DBS
umol/L
16705-6
Cystathionine [Presence] in Urine
53069-1
Cystathionine [Moles/volume] in Body fluid
umol/L
25887-1
Cystathionine [Moles/volume] in 24 hour Urine
umol/L
27349-0
Cystathionine [Presence] in 24 hour Urine
30070-7
Cystathionine [Moles/volume] in Amniotic fluid
umol/dL
32236-2
Cystathionine [Moles/volume] in Vitreous fluid
umol/L

Available Lab Tests

Order tests that measure this biomarker

Amino Acids (Plasma)

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