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Updated: May 1, 2026

HPLC Measurement of the DNA Oxidation Biomarker, 8-oxo-7,8-dihydro-2’-deoxyguanosine, in Cultured Cells and Animal Tissues

HPLC Measurement of the DNA Oxidation Biomarker, 8-oxo-7,8-dihydro-2’-deoxyguanosine, in Cultured Cells and Animal Tissues

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4-hydroxyglutamate is a biomarker for primary hyperoxaluria type 3.

James J Pitt¹, Frank Willis, Nicholas Tzanakos

¹Victorian Clinical Genetics Services, Murdoch Childrens Research Institute, Melbourne, Australia, james.pitt@vcgs.org.au.

|February 25, 2014

Summary

Primary hyperoxaluria type 3 (PH3) is a metabolic disorder causing kidney stones. Measuring 4-hydroxyglutamate (4OHGlu) in urine offers a new biochemical screening method for PH3, aiding in early diagnosis.

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Area of Science:

Biochemistry
Genetics
Metabolic Disorders

Background:

Primary hyperoxaluria type 3 (PH3) is a genetic disorder affecting 4-hydroxyproline metabolism.
Current PH3 diagnosis relies on genetic mutation detection.
PH3 leads to kidney stone disease due to altered metabolism.

Purpose of the Study:

To investigate 4-hydroxyglutamate (4OHGlu) excretion as a biomarker for PH3.
To establish a biochemical screening method for PH3.
To assess the utility of 4OHGlu measurement in diagnosing PH3.

Main Methods:

Flow injection tandem mass spectrometry was used to measure urinary 4OHGlu levels.
4OHGlu levels were analyzed in PH3 patients, their parents, and healthy controls.
Age-specific reference ranges for 4OHGlu were established.

Main Results:

Urinary 4OHGlu levels were significantly elevated in PH3 patients compared to controls.
Carrier parents showed moderately increased 4OHGlu levels.
4OHGlu measurement successfully diagnosed PH3 prospectively in an infant and was detected in a newborn blood spot.

Conclusions:

Urinary 4-hydroxyglutamate (4OHGlu) is a reliable biomarker for Primary hyperoxaluria type 3 (PH3).
4OHGlu testing provides a robust, high-throughput biochemical screen for PH3.
This method facilitates early diagnosis and management of PH3-related kidney stone disease.