Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy

This study investigated the therapeutic potential of the nuclear retinoid X receptor (RXR) in mitigating the progression of alpha-synucleinopathies (αSNPs), particularly in Parkinson’s disease (PD). PD-like pathology in mice was successfully induced through the co-delivery of AAV expressing human α-...

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Main Authors: Assylbek Zhylkibayev, Christopher R. Starr, M. Iqbal Hossain, Sandeep Kumar, Shaida A. Andrabi, Maria B. Grant, Venkatram R. Atigadda, Marina S. Gorbatyuk, Oleg S. Gorbatyuk
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Language:English
Published: MDPI AG 2025-05-01
Series:Cells
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Online Access:https://www.mdpi.com/2073-4409/14/10/685
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author Assylbek Zhylkibayev
Christopher R. Starr
M. Iqbal Hossain
Sandeep Kumar
Shaida A. Andrabi
Maria B. Grant
Venkatram R. Atigadda
Marina S. Gorbatyuk
Oleg S. Gorbatyuk
author_facet Assylbek Zhylkibayev
Christopher R. Starr
M. Iqbal Hossain
Sandeep Kumar
Shaida A. Andrabi
Maria B. Grant
Venkatram R. Atigadda
Marina S. Gorbatyuk
Oleg S. Gorbatyuk
author_sort Assylbek Zhylkibayev
collection DOAJ
description This study investigated the therapeutic potential of the nuclear retinoid X receptor (RXR) in mitigating the progression of alpha-synucleinopathies (αSNPs), particularly in Parkinson’s disease (PD). PD-like pathology in mice was successfully induced through the co-delivery of AAV expressing human α-synuclein (αS) and αS preformed fibrils (PFFs) into the substantia nigra pars compacta (SNpc). Significant increases in Lewy body (LB)-like inclusions, loss of tyrosine hydroxylase-positive (TH+) neurons, and reductions in dopamine (DA) levels in the striatum were observed. Additionally, diminished levels of PPARα and NURR1—proteins essential for neuronal survival—along with elevated expression of IBA1 and GFAP, markers of microglial activation and astrocytic gliosis, respectively, are associated with the pathogenesis of Parkinson’s disease. AAV-mediated overexpression of human RXRα demonstrated preservation of TH+ neurons, prevention of DA decline, and attenuation of αS accumulation. Furthermore, RXR-treated PD brains showed a reduced number of GFAP+ and Iba1+ cells, decreased GFAP+ and IBA1+ immunoreactivity, and fewer and less widespread LB-like aggregates. RXR overexpression also enhanced the production of PPARα and NURR1. These findings suggest that RXRα upregulation promotes neuroprotection by mitigating αSNPs and chronic neuroinflammation, a major contributor to PD progression. This research underscores the therapeutic potential of targeting nuclear receptors, such as RXR, in neurodegenerative diseases like PD.
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spelling doaj-art-60abd6cd488a401490df17d42fba8a212025-08-20T03:47:52ZengMDPI AGCells2073-44092025-05-01141068510.3390/cells14100685Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-SynucleinopathyAssylbek Zhylkibayev0Christopher R. Starr1M. Iqbal Hossain2Sandeep Kumar3Shaida A. Andrabi4Maria B. Grant5Venkatram R. Atigadda6Marina S. Gorbatyuk7Oleg S. Gorbatyuk8Department of Biochemistry, School of Medicine, Wake Forest University, Winston-Salem, NC 27157, USADepartment of Ophthalmology, School of Medicine, University of Alabama, Birmingham, AL 35233, USADepartment of Pharmacology and Toxicology, School of Medicine, University of Alabama, Birmingham, AL 35294, USADepartment of Biochemistry, School of Medicine, Wake Forest University, Winston-Salem, NC 27157, USADepartment of Pharmacology and Toxicology, School of Medicine, University of Alabama, Birmingham, AL 35294, USADepartment of Ophthalmology, School of Medicine, University of Alabama, Birmingham, AL 35233, USADepartment of Dermatology, Heersink School of Medicine, University of Alabama, Birmingham, AL 35233, USADepartment of Biochemistry, School of Medicine, Wake Forest University, Winston-Salem, NC 27157, USADepartment of Translational Neuroscience, School of Medicine, Wake Forest University, Winston-Salem, NC 27157, USAThis study investigated the therapeutic potential of the nuclear retinoid X receptor (RXR) in mitigating the progression of alpha-synucleinopathies (αSNPs), particularly in Parkinson’s disease (PD). PD-like pathology in mice was successfully induced through the co-delivery of AAV expressing human α-synuclein (αS) and αS preformed fibrils (PFFs) into the substantia nigra pars compacta (SNpc). Significant increases in Lewy body (LB)-like inclusions, loss of tyrosine hydroxylase-positive (TH+) neurons, and reductions in dopamine (DA) levels in the striatum were observed. Additionally, diminished levels of PPARα and NURR1—proteins essential for neuronal survival—along with elevated expression of IBA1 and GFAP, markers of microglial activation and astrocytic gliosis, respectively, are associated with the pathogenesis of Parkinson’s disease. AAV-mediated overexpression of human RXRα demonstrated preservation of TH+ neurons, prevention of DA decline, and attenuation of αS accumulation. Furthermore, RXR-treated PD brains showed a reduced number of GFAP+ and Iba1+ cells, decreased GFAP+ and IBA1+ immunoreactivity, and fewer and less widespread LB-like aggregates. RXR overexpression also enhanced the production of PPARα and NURR1. These findings suggest that RXRα upregulation promotes neuroprotection by mitigating αSNPs and chronic neuroinflammation, a major contributor to PD progression. This research underscores the therapeutic potential of targeting nuclear receptors, such as RXR, in neurodegenerative diseases like PD.https://www.mdpi.com/2073-4409/14/10/685α-synucleinopathyParkinson’s diseaseretinoid X receptorα-synucleinLewy bodydopamine
spellingShingle Assylbek Zhylkibayev
Christopher R. Starr
M. Iqbal Hossain
Sandeep Kumar
Shaida A. Andrabi
Maria B. Grant
Venkatram R. Atigadda
Marina S. Gorbatyuk
Oleg S. Gorbatyuk
Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
Cells
α-synucleinopathy
Parkinson’s disease
retinoid X receptor
α-synuclein
Lewy body
dopamine
title Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
title_full Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
title_fullStr Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
title_full_unstemmed Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
title_short Retinoid X Receptor as a Therapeutic Target to Treat Neurological Disorders Associated with α-Synucleinopathy
title_sort retinoid x receptor as a therapeutic target to treat neurological disorders associated with α synucleinopathy
topic α-synucleinopathy
Parkinson’s disease
retinoid X receptor
α-synuclein
Lewy body
dopamine
url https://www.mdpi.com/2073-4409/14/10/685
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