The role of Nurr1 in the development of dopaminergic neurons and Parkinson's disease
Abstract
Nurr1, a transcription factor belonging to the orphan nuclear receptor superfamily, is critical in the development and maintenance of the dopaminergic system and as such it may have role in the pathogenesis of Parkinson' disease (PD). Human Nurr1 gene has been mapped to chromosome 2q22-23 and Nurr1 protein is predominantly expressed in central dopaminergic neurons. Nurr1 interacts with other factors critical for the survival of mensencephalic dopaminergic neurons and it appears to regulate the expression of tyrosine hydroxylase (TH), dopamine transporter (DAT), vesicular monoamine transporter 2 (VMAT2), and l-aromatic amino acid decarboxylase (AADC), all of which are important in the synthesis and storage of dopamine. Experimental studies in Nurr1 knock-out mice indicate that Nurr1 deficiency results in impaired dopaminergic function and increased vulnerability of those midbrain dopaminergic neurons that degenerate in PD. Decreased Nurr1 expression is found in the autopsied PD midbrains, particularly in neurons containing Lewy bodies, as well as in peripheral lymphocytes of patients with parkinsonian disorders. Several variants in Nurr1 gene have been reported in association with PD. All these studies suggest that Nurr1 is not only essential in the development of mensencephalic dopaminergic neurons and maintenance of their functions, but it may also play a role in the pathogenesis of PD.
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In experimental models of PD, RA protected against neurodegeneration of midbrain dopaminergic neurons in the substantia nigra.
“In experimental models of PD, RA protected against neurodegeneration of midbrain dopaminergic neurons in the substantia nigra86,87.”
From Advances and challenges in retinoid delivery systems in regenerative and therapeutic medicine (2020) · cites this paper as [87]
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Self-citation
- Authors
- Jankovic, J., Chen, S., & Le, W. D.
- Affiliations
- Baylor College of Medicine (US)
- Shanghai Jiao Tong University (CN)
- Corresponding author
- Le, W.D.
- Venue
- Progress in Neurobiology
- Publisher
- Elsevier BV
- Published
- 2005-09
- Volume
- 77
- Issue
- 1-2
- Pages
- 128-138
- ISSN
- 0301-0082 (ISSN-L)
- OpenAlex
- W2048841722
- Cited by
- 325
- References
- 89
- Citations by year
- 2026: 10 · 2025: 8 · 2024: 15 · 2023: 14 · 2022: 21 · 2021: 22 · 2020: 13 · 2019: 19 · 2018: 13 · 2017: 19 · 2016: 15 · 2015: 18 · 2014: 16 · 2013: 22 · 2012: 16
- FWCI
- 1.06
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Progress in Neurobiology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health
- National Institute of Neurological Disorders and Stroke (NS043567, R01NS043567, NS40370, R01NS040370)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Nuclear Receptors and Signaling
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neurotransmitter Receptor Influence on Behavior
- Keywords
- Nurr1, Parkinson's disease, dopaminergic neurons, midbrain, transcription factors, orphan nuclear receptor, tyrosine hydroxylase, dopamine transporter, vesicular monoamine transporter 2, aromatic L-amino acid decarboxylase, Lewy bodies, dopaminergic neuron development, dopaminergic neuron survival
- MeSH terms
- Animals, Brain, Cell Differentiation, DNA-Binding Proteins, Dopamine, Humans, Neurons, Parkinson Disease, Transcription Factors, Signal Transduction, Nuclear Receptor Subfamily 4, Group A, Member 2
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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