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Cystathionine Levels in Patients With Huntington Disease

N A Aziz1, W Onkenhout2, H J Kerstens2

  • 1Department of Neurology, Leiden University Medical Centre, Leiden, the Netherlands.

Plos Currents
|October 6, 2015
PubMed

Insights

Plasma and urine cystathionine levels did not differ between early-stage Huntington disease (HD) patients and controls. These findings suggest cystathionine is not a useful biomarker for HD progression.

Area of Science:

  • Biochemistry
  • Neuroscience
  • Genetics

Background:

  • Huntington disease (HD) is associated with reduced cystathionine γ-lyase (CSE) activity.
  • CSE is crucial for cysteine synthesis from cystathionine.

Purpose of the Study:

  • To investigate if blood and urine cystathionine levels are elevated in HD patients.
  • To determine if cystathionine levels correlate with HD progression.

Main Methods:

  • Measured cystathionine and 22 other amino acids in plasma and urine.
  • Included nine early-stage HD patients and nine matched controls.

Main Results:

  • No significant differences in plasma or urine cystathionine concentrations were observed between HD patients and controls.
  • No significant differences were found for any other measured amino acids.

Conclusions:

  • Plasma and urine cystathionine levels do not change in early-stage HD.
  • Cystathionine is unlikely to serve as a reliable biomarker for Huntington disease.
Abstract

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