Spinocerebellar ataxias (SCAs) are a large and diverse group of autosomal-dominant neurodegenerative diseases. No drugs have been approved for these relentlessly progressive and fatal SCAs. Our previous studies indicate that oxidative stress, neuroinflammation, and neuronal apoptosis are elevated in the SCA17 mice, which are the main therapeutic targets of hyperbaric oxygen treatment (HBOT). HBOT is considered to be an alternative and less invasive therapy for SCAs. In this study, we evaluated the HBOT (2.2 ATA for 14 days) effect and the persistence for the management of SCA17 mice and their wild-type littermates. We found HBOT attenuated the motor coordination and cognitive impairment of SCA17 mice and which persisted for about 1 month after the treatment. The results of several biochemistry and liver/kidney hematoxylin and eosin staining show the HBOT condition has no obvious toxicity in the mice. Immunostaining analyses show that the neuroprotective effect of HBOT could be through the promotion of BDNF production and the amelioration of neuroinflammation. Surprisingly, HBOT executes different effects on the male and female SCA17 mice, including the reduction of neuroinflammation and activation of CaMKII and ERK. This study suggests HBOT is a potential alternative therapeutic treatment for SCA17. Accumulated findings have revealed the similarity in disease pathomechanisms and possible therapeutic strategies in polyQ diseases; therefore, HBOT could be an optional treatment as well as the other polyQ diseases.
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http://dx.doi.org/10.1007/s12311-023-01548-y | DOI Listing |
Appl Radiat Isot
March 2025
Department of Isotope Application Research, National Atomic Research Institute, Taoyuan City, Taiwan, ROC.
Histone deacetylase 6 (HDAC6) is an enzyme crucial in epigenetic regulation and protein degradation, with implications in various cancers and neurodegenerative disorders. While HDAC6 is recognized as a promising therapeutic target for Parkinson's and Alzheimer's diseases, its involvement in spinocerebellar ataxias (SCAs) remains underexplored. Currently, there are no direct methods available for characterizing HDAC6 in the brains of living subjects.
View Article and Find Full Text PDFHum Genet
December 2023
Instituto de Investigação e Inovação em Saúde (i3S), 4200-135, Porto, Portugal.
Polyglutamine (polyQ) spinocerebellar ataxias (SCAs) comprise a group of autosomal dominant neurodegenerative disorders caused by (CAG/CAA) expansions. The elongated stretches of adjacent glutamines alter the conformation of the native proteins inducing neurotoxicity, and subsequent motor and neurological symptoms. Although the etiology and neuropathology of most polyQ SCAs have been extensively studied, only a limited selection of therapies is available.
View Article and Find Full Text PDFCerebellum
April 2024
Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
Spinocerebellar ataxias (SCAs) are a large and diverse group of autosomal-dominant neurodegenerative diseases. No drugs have been approved for these relentlessly progressive and fatal SCAs. Our previous studies indicate that oxidative stress, neuroinflammation, and neuronal apoptosis are elevated in the SCA17 mice, which are the main therapeutic targets of hyperbaric oxygen treatment (HBOT).
View Article and Find Full Text PDFJ Neurosci
June 2021
Institut de Génétique et de Biologie Moléculaire et Cellulaire, Illkirch 67404, France
Neurosci Lett
November 2020
Department of Life Science, National Taiwan Normal University, Taiwan. Electronic address:
Spinocerebellar ataxia type 17 (SCA17) is an autosomal dominant neurodegenerative disease caused by CAG expansion in the gene encoding the TATA-binding protein (TBP). The neurological features of SCA17 are Purkinje cell loss and gliosis. We have generated SCA17 transgenic mice which recapitulate the patients' phenotypes and are suitable for the study of the SCA17 pathomechanism.
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