ReviewInternational journal of clinical and experimental pathology2011
Pathological accumulation of atrophin-1 in dentatorubralpallidoluysian atrophy.
Review in International journal of clinical and experimental pathology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
What it found
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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.
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Who cites it
15 citing papers in PubMed, 23 citations in OpenAlex.
- Accurate Quantification of Mutant and Wild-Type polyQ Proteins Using Simple Western Capillary Immunoassays.Molecular neurobiology · 2025Article
- Dentatorubral-Pallidoluysian Atrophy (DRPLA) in Three Successive Generations with Anticipation in an Indian Family.Annals of Indian Academy of Neurology · 2025Article
- Differential microRNA expression in the SH-SY5Y human cell model as potential biomarkers for Huntington's disease.Frontiers in cellular neuroscience · 2024Article
- Case report: Corneal endothelial degeneration and optic atrophy in dentatorubral-pallidoluysian atrophy quantified by specular micrography and optical coherence tomography.Frontiers in neurology · 2022Article
- Nucleophagy: from homeostasis to disease.Cell death and differentiation · 2019Review
- Identification of Candidate Signature Genes and Key Regulators Associated With Trypanotolerance in the Sheko Breed.Frontiers in genetics · 2019Article
- Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy.Journal of Korean medical science · 2018Article
- Successful treatment of psychosis in dentatorubral-pallidoluysian atrophy with quetiapine: A case report.Neuropsychopharmacology reports · 2018Article
- Roles of Post-translational Modifications in Spinocerebellar Ataxias.Frontiers in cellular neuroscience · 2018Review
- Intrinsic Disorder in Proteins with Pathogenic Repeat Expansions.Molecules (Basel, Switzerland) · 2017Review
- Common disease signatures from gene expression analysis in Huntington's disease human blood and brain.Orphanet journal of rare diseases · 2016Article
- Epigenetic regulation of Atrophin1 by lysine-specific demethylase 1 is required for cortical progenitor maintenance.Nature communications · 2014Article
- A multidimensional approach to an in-depth proteomics analysis of transcriptional regulators in neuroblastoma cells.Journal of neuroscience methods · 2013Article
- The oncoprotein BCL11A binds to orphan nuclear receptor TLX and potentiates its transrepressive function.PloS one · 2012Article
- Establishing resources and increasing awareness to advance research on Dentatorubral-pallidoluysian atrophy toward a treatment: a patient organization perspective.Therapeutic advances in rare diseaseReview
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Dentatorubral-pallidoluysian atrophy (DRPLA) is caused by the expansion of polyglutamine (polyQ) in atrophin-1 (ATN1), also known as DRPLA protein. ATN1 is ubiquitously expressed in the central nervous system (CNS), although selective regions of CNS are degenerated in DRPLA, and this selective neuronal damage gives rise to the specific clinical features of DRPLA. Accumulation of mutant ATN1 that carries an expanded polyQ tract seems to be the primary cause of DRPLA neurodegeneration, but it is still unclear how the accumulation of ATN1 leads to neu-rodegeneration. Recently, cleaved fragments of ATN1 were shown to accumulate in the disease models and the brain tissues of patients with DRPLA. Furthermore, proteolytic processing of ATN1 may regulate the intracellular localization of ATN1 and its fragments. Therefore, proteolytic processing of ATN1 may provide clues to disease pathogenesis and hopefully aid in the determination of molecular targets for effective therapeutic approaches for DRPLA.
Indexed as
Identifiers
21577324PMC3093063W17603606What 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.