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Cited by 176

The DOI matches its Crossref record; 13 of the 13 references it cites were found in a registry or an index.

Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease

Open access
Schwarcz, Robert, Guidetti, Paolo, Sathyasaikumar, Korrapati V., Muchowski, Paul J.·2010·Progress in Neurobiology
DOI 10.1016/j.pneurobio.2009.04.005
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Abstract

The neurodegenerative disease Huntington’s Disease (HD) is caused by an expanded polyglutamine (polyQ) tract in the protein huntingtin (htt). Although the gene encoding htt was identified and cloned more than 15 years ago, and in spite of impressive efforts to unravel the mechanism(s) by which mutant htt induces nerve cell death, these studies have so far not led to a good understanding of pathophysiology or an effective therapy. Set against a historical background, we review data supporting the idea that metabolites of the kynurenine pathway (KP) of tryptophan degradation provide a critical link between mutant htt and the pathophysiology of HD. New studies in HD brain and genetic model organisms suggest that the disease may in fact be causally related to early abnormalities in KP metabolism, favoring the formation of two neurotoxic metabolites, 3-hydroxykynurenine and quinolinic acid, over the related neuroprotective agent kynurenic acid. These findings not only link the excitotoxic hypothesis of HD pathology to an impairment of the KP but also define new drug targets and therefore have direct therapeutic implications. Thus, pharmacological normalization of the imbalance in brain KP metabolism may provide clinical benefits, which could be especially effective in the early stages of the disease.

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Metadata

Authors
Schwarcz, R., Guidetti, P., Sathyasaikumar, K. V., & Muchowski, P. J.
Venue
Progress in Neurobiology
Publisher
Elsevier BV
Published
2010-02
Volume
90
Issue
2
Pages
230-245
ISSN
0301-0082 (ISSN-L)
DOI
10.1016/j.pneurobio.2009.04.005
OpenAlex
W1982792716
Cited by
176
References
13
FWCI
6.77
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease. https://app.cochat.ai/papers/10.1016/j.pneurobio.2009.04.005
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Schwarcz, R., Guidetti, P., Sathyasaikumar, K. V., & Muchowski, P. J. (2010). Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease. Progress in Neurobiology. https://doi.org/10.1016/j.pneurobio.2009.04.005

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.