ArticleProceedings of the National Academy of Sciences of the United States of America2006
Deficiency of the zinc finger protein ZPR1 causes neurodegeneration.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 1 of them a synthesis that pooled it.
What it found
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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
31 citing papers in PubMed, 1 synthesis or guideline pooled it, 50 citations in OpenAlex.
- CADM1 is a strong neuroblastoma candidate gene that maps within a 3.72 Mb critical region of loss on 11q23.BMC cancer · 2008Pooled it
- Survival motor neuron protein-independent amelioration of spinal muscular atrophy by pharmacological inhibition of c-Jun-NHBrain communications · 2026Article
- The human zinc-binding cysteine proteome.Cell · 2025Article
- Role of senataxin in R-loop-mediated neurodegeneration.Brain communications · 2024Review
- Zinc-finger protein Zpr1 is a bespoke chaperone essential for eEF1A biogenesis.Molecular cell · 2023Article
- Pan-Cancer Profiling and Digital Pathology Analysis Reveal Negative Prognostic Biomarker ZPR1 Associated with Immune Infiltration and Treatment Response in Hepatocellular Carcinoma.Journal of hepatocellular carcinoma · 2023Article
- Review
- Zinc finger protein ZPR1: promising survival motor neuron protein-dependent modifier for the rescue of spinal muscular atrophy.Neural regeneration research · 2022Article
- Mutation in senataxin alters the mechanism of R-loop resolution in amyotrophic lateral sclerosis 4.Brain : a journal of neurology · 2022Article
- R-loop Mediated DNA Damage and Impaired DNA Repair in Spinal Muscular Atrophy.Frontiers in cellular neuroscience · 2022Review
- High-fat diet-associated cognitive decline: Is zinc finger protein 1 (ZPR1) the molecular connection?Current research in physiology · 2021Review
- ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy.Brain : a journal of neurology · 2020Article
- ZPR1-Dependent Neurodegeneration Is Mediated by the JNK Signaling Pathway.Journal of experimental neuroscience · 2019Article
- Molecular Factors Involved in Spinal Muscular Atrophy Pathways as Possible Disease-modifying Candidates.Current genomics · 2018Review
- Deregulation of ZPR1 causes respiratory failure in spinal muscular atrophy.Scientific reports · 2017Article
- Associations of genetic variants for adult lipid levels with lipid levels in children. The Generation R Study.Journal of lipid research · 2016Article
- Molecular Mechanisms of Neurodegeneration in Spinal Muscular Atrophy.Journal of experimental neuroscience · 2016Review
- Genetic inhibition of JNK3 ameliorates spinal muscular atrophy.Human molecular genetics · 2015Article
- Article
- Spinal muscular atrophy and the antiapoptotic role of survival of motor neuron (SMN) protein.Molecular neurobiology · 2013Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations that cause reduced expression of the full-length Survival Motor Neurons (SMN) protein are a major cause of spinal muscular atrophy (SMA), a disease characterized by degeneration of the alpha-motor neurons in the anterior horn of the spinal cord. The severity of SMA may be influenced by the actions of modifier genes. One potential modifier gene is represented by ZPR1, which is down-regulated in patients with SMA and encodes a zinc finger protein that interacts with complexes formed by SMN. To test the functional significance of ZPR1 gene down-regulation, we examined a mouse model with targeted ablation of the Zpr1 gene. We report that ZPR1-deficient mice exhibit axonal pathology and neurodegeneration. These data identify ZPR1 deficiency as a contributing factor in neurodegenerative disorders.
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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.