ArticleCurrent trends in neurology2011
The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology.
Article in Current trends in neurology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 121 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
121 citing papers in PubMed, 207 citations in OpenAlex.
- TRiC/CCT chaperonin governs RNA polymerase II activity in the nucleus to support RNA homeostasis.Molecular cell · 2026Article
- What can clinical disorders involving the basal ganglia tell us about the pathophysiology of obsessive and compulsive symptoms?Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Review
- Cortistatin as a modulator of inflammatory and mitochondrial dysfunction in Huntington´s disease.Journal of neuroinflammation · 2026Article
- HDDF2-A Novel Patient-Derived Fibroblast Line from Huntington's Disease with Prominent Cellular Senescence and polyQ Pathology.Biomedicines · 2026Article
- Insights into neurodevelopmental features of Huntington's disease from stem cell-derived models including organoids.Journal of Huntington's disease · 2026Review
- Aberrant medial ganglionic eminence (MGE) GABAergic neurogenesis contributes to Huntington's disease pathogenesis.Neurobiology of disease · 2026Article
- Astrocyte heterogeneity and gliosis in Huntington's disease: Histopathological insights into striatal and white matter pathology.Histology and histopathology · 2026Review
- Mutant huntingtin expression in somatostatin-positive interneurons contributes to neurophysiological and behavioral phenotypes in BACHD mice.Disease models & mechanisms · 2026Article
- Are neurodegenerative diseases late-onset neurodevelopmental disorders? Tracing the developmental origins of neuronal vulnerability.Frontiers in neuroscience · 2026Review
- Metabolomic analysis of the endometrium of Large White and Meishan pigs reveals differences in biological processes during late gestation.BMC genomics · 2025Article
- Article
- Methylene Blue Attenuates 3-Nitropropionic Acid-Induced Oxidative Stress and Mitochondrial Dysfunction in Striatal Cells: Therapeutic Implications in Huntington's Disease Neuropathology.International journal of molecular sciences · 2025Article
- CRISPR-based gene therapy for huntington's disease: current advances and future prospects.Neurogenetics · 2025Review
- Accurate Quantification of Mutant and Wild-Type polyQ Proteins Using Simple Western Capillary Immunoassays.Molecular neurobiology · 2025Article
- Revolutionizing Huntington's Disease Treatment: Breakthroughs in AAV-Mediated Gene Therapy.Cells · 2025Review
- AI-Enhanced Transcriptomic Discovery of Druggable Targets and Repurposed Therapies for Huntington's Disease.Brain sciences · 2025Review
- Mutant THAP11 causes cerebellar neurodegeneration and triggers TREM2-mediated microglial activation in mice.The Journal of clinical investigation · 2025Article
- The Role of MicroRNAs in Neurodegeneration: Insights from Huntington's Disease.Molecular neurobiology · 2025Review
- DurableMolecular therapy. Nucleic acids · 2025Article
61 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Huntington's Disease is an adult-onset dominant heritable disorder characterized by progressive psychiatric disruption, cognitive deficits, and loss of motor coordination. It is caused by expansion of a polyglutamine tract within the N-terminal domain of the Huntingtin protein. The mutation confers a toxic gain-of-function phenotype, resulting in neurodegeneration that is most severe in the striatum. Increasing experimental evidence from genetic model systems such as mice, zebrafish, and Drosophila suggest that polyglutamine expansion within the Huntingtin protein also disrupts its normal biological function. Huntingtin is widely expressed during development and has a complex and dynamic distribution within cells. It is predicted to be a protein of pleiotropic function, interacting with a large number of effector proteins to mediate a host of physiological processes. In this review, we highlight the wildtype function of Huntingtin, focusing on its postdevelopmental roles in axonal trafficking, regulation of gene transcription, and cell survival. We then discuss how potential loss-of-function phenotypes resulting in polyglutamine expansion within Huntingtin may have direct relevance to the underlying pathophysiology of Huntington's Disease.
Identifiers
22180703PMC3237673W2139123778What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.