ArticleProceedings of the National Academy of Sciences of the United States of America2006
Slow progressive degeneration of nigral dopaminergic neurons in postnatal Engrailed mutant mice.
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 65 papers.
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Who cites it
65 citing papers in PubMed.
- Modulation of WNT and FGF18 enhances yield and subtype identity of hPSC-derived midbrain dopamine neurons.The Journal of clinical investigation · 2026Article
- Engrailed 1 promotes immune evasion and chemoresistance in glioma through single cell and CeRNA network analyses.Scientific reports · 2026Article
- Unravelling axonal transcriptional landscapes: insights from induced pluripotent stem cell-derived cortical neurons and implications for motor neuron degeneration.Open biology · 2025Article
- Conserved roles of engrailed: patterning tissues and specifying cell types.Development (Cambridge, England) · 2024Review
- Engrailed transcription factors direct excitatory cerebellar neuron diversity and survival.Development (Cambridge, England) · 2024Article
- Cannabinoid type-1 receptor signaling in dopaminergic Engrailed-1 expressing neurons modulates motivation and depressive-like behavior.Frontiers in molecular neuroscience · 2024Article
- Nigral transcriptomic profiles in Engrailed-1 hemizygous mouse models of Parkinson's disease reveal upregulation of oxidative phosphorylation-related genes associated with delayed dopaminergic neurodegeneration.Frontiers in aging neuroscience · 2024Article
- ENGRAILED-1 transcription factor has a paracrine neurotrophic activity on adult spinal α-motoneurons.EMBO reports · 2023Article
- Robust derivation of transplantable dopamine neurons from human pluripotent stem cells by timed retinoic acid delivery.Nature communications · 2022Article
- Review
- An Update on the Molecular Mechanism of the Vertebrate Isthmic Organizer Development in the Context of the Neuromeric Model.Frontiers in neuroanatomy · 2022Review
- Developmental pathways linked to the vulnerability of adult midbrain dopaminergic neurons to neurodegeneration.Frontiers in molecular neuroscience · 2022Review
- Article
- Genetic targeting of adult Renshaw cells using a Calbindin 1 destabilized Cre allele for intersection with Parvalbumin or Engrailed1.Scientific reports · 2021Article
- Morphological alterations of the reticular thalamic nucleus in Engrailed-2 knockout mice.Journal of anatomy · 2020Article
- Current Status of Stem Cell-Derived Therapies for Parkinson's Disease: From Cell Assessment and Imaging Modalities to Clinical Trials.Frontiers in neuroscience · 2020Review
- Crosstalk of Intercellular Signaling Pathways in the Generation of Midbrain Dopaminergic Neurons In Vivo and from Stem Cells.Journal of developmental biology · 2019Review
- Loss of One Engrailed1 Allele Enhances Induced α-Synucleinopathy.Journal of Parkinson's disease · 2019Article
- Axon-Seq Decodes the Motor Axon Transcriptome and Its Modulation in Response to ALS.Stem cell reports · 2018Article
- Identification of Multiple QTLs Linked to Neuropathology in the Engrailed-1 Heterozygous Mouse Model of Parkinson's Disease.Scientific reports · 2016Article
5 more citing papers are in PubMed but not listed here.
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Authors and funding
9 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The homeobox transcription factors Engrailed-1 and Engrailed-2 are required for the survival of mesencephalic dopaminergic neurons in a cell-autonomous and gene-dose-dependent manner. Because of this requirement, the cells die by apoptosis when all four alleles of the Engrailed genes are genetically ablated (En1-/-;En2-/-). In the present study, we show that viable and fertile mice, heterozygous null for Engrailed-1 and homozygous null for Engrailed-2 (En1+/-;En2-/-), have an adult phenotype that resembles key pathological features of Parkinson's disease. Specifically, postnatal mutant mice exhibit a progressive degeneration of dopaminergic neurons in the substantia nigra during the first 3 mo of their lives, leading to diminished storage and release of dopamine in the caudate putamen, motor deficits similar to akinesia and bradykinesia, and a lower body weight. This genetic model may provide access to the molecular etiology for Parkinson's disease and could assist in the development of novel treatments for this neurodegenerative disorder.
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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.