Brain urea increase is an early Huntington’s disease pathogenic event observed in a prodromal transgenic sheep model and HD cases Article Swipe
Renée R. Handley
,
Suzanne J. Reid
,
Rüdiger Bräuning
,
Paul Maclean
,
Emily Mears
,
Imche Fourie
,
Stefano Patassini
,
Garth J. S. Cooper
,
Skye R. Rudiger
,
Clive J. McLaughlan
,
Paul J. Verma
,
James F. Gusella
,
Marcy E. MacDonald
,
Henry J. Waldvogel
,
C. Simon Bawden
,
Richard L. M. Faull
,
Russell G. Snell
·
YOU?
·
· 2017
· Open Access
·
· DOI: https://doi.org/10.1073/pnas.1711243115
YOU?
·
· 2017
· Open Access
·
· DOI: https://doi.org/10.1073/pnas.1711243115
Significance We present evidence for the presymptomatic dysregulation of urea metabolism in Huntington’s disease (HD). We identified increased levels of a urea transporter transcript and other osmotic regulators in the striatum of our prodromal sheep model of HD and a concomitant increase in striatal and cerebellar urea. Elevated urea was also detected in brain tissue from postmortem HD cases, including cases with low-level cell loss, implying that increased brain urea in HD is not just a product of end-stage cachexia. Disruption of urea metabolism is known to cause neurologic impairment and could initiate neurodegeneration and the symptoms of HD. Our findings suggest that lowering brain levels of urea and/or ammonia would be a worthwhile therapeutic target in HD.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1073/pnas.1711243115
- https://www.pnas.org/content/pnas/114/52/E11293.full.pdf
- OA Status
- bronze
- Cited By
- 104
- References
- 67
- Related Works
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- OpenAlex ID
- https://openalex.org/W2774354480
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W2774354480Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1073/pnas.1711243115Digital Object Identifier
- Title
-
Brain urea increase is an early Huntington’s disease pathogenic event observed in a prodromal transgenic sheep model and HD casesWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2017Year of publication
- Publication date
-
2017-12-11Full publication date if available
- Authors
-
Renée R. Handley, Suzanne J. Reid, Rüdiger Bräuning, Paul Maclean, Emily Mears, Imche Fourie, Stefano Patassini, Garth J. S. Cooper, Skye R. Rudiger, Clive J. McLaughlan, Paul J. Verma, James F. Gusella, Marcy E. MacDonald, Henry J. Waldvogel, C. Simon Bawden, Richard L. M. Faull, Russell G. SnellList of authors in order
- Landing page
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https://doi.org/10.1073/pnas.1711243115Publisher landing page
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https://www.pnas.org/content/pnas/114/52/E11293.full.pdfDirect link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
bronzeOpen access status per OpenAlex
- OA URL
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https://www.pnas.org/content/pnas/114/52/E11293.full.pdfDirect OA link when available
- Concepts
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Huntington's disease, Urea, Neurodegeneration, Striatum, Urea cycle, Endocrinology, Internal medicine, Disease, Biology, Medicine, Biochemistry, Arginine, Dopamine, Amino acidTop concepts (fields/topics) attached by OpenAlex
- Cited by
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104Total citation count in OpenAlex
- Citations by year (recent)
-
2025: 11, 2024: 15, 2023: 18, 2022: 18, 2021: 20Per-year citation counts (last 5 years)
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67Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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| publication_year | 2017 |
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