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Updated: Aug 26, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
Published on: February 27, 2018
Hyperhomocysteinaemia in treated patients with Huntington's disease homocysteine in HD
Jürgen Andrich1, Carsten Saft, Anneliese Arz
1Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany.
Insights
Elevated total homocysteine (t-Hcys) levels are found in treated Huntington
Area of Science:
- Neuroscience
- Biochemistry
- Genetics
Background:
- Huntington disease (HD) is a neurodegenerative disorder.
- The protein Huntingtin plays a role in homocysteine metabolism.
- Elevated homocysteine levels are implicated in neurological conditions.
Purpose of the Study:
- To investigate the association between total homocysteine (t-Hcys) levels and Huntington disease (HD).
- To explore the potential role of homocysteine in HD-related neurodegeneration.
Main Methods:
- Comparison of plasma t-Hcys levels in treated HD patients, untreated HD subjects, and control groups.
- Analysis of the interaction between Huntingtin protein and cystathionine beta-synthase.
Main Results:
- Treated HD patients exhibited significantly higher plasma t-Hcys levels than controls and untreated HD subjects.
- The Huntingtin protein is known to interact with cystathionine beta-synthase, an enzyme crucial for homocysteine metabolism.
Conclusions:
- Increased t-Hcys levels in treated HD patients suggest a potential link between homocysteine and disease progression.
- Homocysteine may contribute to neurodegeneration in Huntington disease.
Abstract:
Significantly increased plasma total homocysteine levels (t-Hcys) appeared in treated Huntington disease (HD) patients compared to controls and untreated HD subjects. Because the protein Huntingtin interacts with the homocysteine metabolism modulating enzyme cystathionine beta-synthase, we hypothesize that homocysteine promotes neurodegeneration in HD.
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