ArticleNeurobiology of disease2006
Genetic interaction between expanded murine Hdh alleles and p53 reveal deleterious effects of p53 on Huntington's disease pathogenesis.
Article in Neurobiology of disease, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 39 citations in OpenAlex.
- p53: from understanding its structure to advances in therapeutic targeting.Signal transduction and targeted therapy · 2026Review
- Oncogenic Pathways in Neurodegenerative Diseases.International journal of molecular sciences · 2022Review
- Huntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells.Molecular neurobiology · 2018Review
- Transcriptional Regulation of the Huntingtin Gene.Journal of Huntington's disease · 2018Review
- Transcriptional Dysregulation and Post-translational Modifications in Polyglutamine Diseases: From Pathogenesis to Potential Therapeutic Strategies.Frontiers in molecular neuroscience · 2018Review
- Possible Role of the Polyglutamine Elongation in Evolution of Amyloid-Related Evolvability.Journal of Huntington's disease · 2018Article
- P53 Dysfunction in Neurodegenerative Diseases - The Cause or Effect of Pathological Changes?Aging and disease · 2017Review
- Is Huntingtin Dispensable in the Adult Brain?Journal of Huntington's disease · 2017Review
- Allele-specific regulation of mutant Huntingtin by Wig1, a downstream target of p53.Human molecular genetics · 2016Article
- Role of p53 in neurodegenerative diseases.Neuro-degenerative diseases · 2012Review
- Genomic analysis of wig-1 pathways.PloS one · 2012Article
- The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease.The Journal of clinical investigation · 2011Review
- Regulation of miR-146a by RelA/NFkB and p53 in STHdh(Q111)/Hdh(Q111) cells, a cell model of Huntington's disease.PloS one · 2011Article
- Huntington's disease and mitochondrial alterations: emphasis on experimental models.Journal of bioenergetics and biomembranes · 2010Article
- Explaining oscillations and variability in the p53-Mdm2 system.BMC systems biology · 2008Article
- Celastrol inhibits polyglutamine aggregation and toxicity though induction of the heat shock response.Journal of molecular medicine (Berlin, Germany) · 2007Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Huntingtin, the protein product of the Huntington's disease (HD) gene, is known to interact with the tumor suppressor p53. It has recently been shown that activation of p53 upregulates the level of huntingtin, both in vitro and in vivo, whereas p53 deficiency in HD-transgenic flies and mice has been found to be beneficial. To explore further the involvement of p53 in HD pathogenesis, we generated mice homozygous for a mutant allele of Hdh (HdhQ140) and with zero, one, or two functional alleles of p53. p53 deficiency resulted in a reduction of mutant huntingtin expression in brain and testis, an increase in proenkephalin mRNA expression and a significant increase in nuclear aggregate formation in the striatum. Because aggregation of mutant huntingtin is suggested to be a protective mechanism, both the increase in aggregate load and the restoration of proenkephalin expression suggest a functional rescue of at least several aspects of the HD phenotype by a deficiency in p53.
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Registered trials
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