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Cerebellum. 2019 Feb;18(1):22-32. doi: 10.1007/s12311-018-0936-3.

Far-infrared Radiation Improves Motor Dysfunction and Neuropathology in Spinocerebellar Ataxia Type 3 Mice.

Author information

1
Vascular and Genomic Center, Changhua Christian Hospital, 135 Nanhsiao Street, Changhua, 50094, Taiwan.
2
Department of Chinese Medicine, China Medical University Hospital, Taichung, 40447, Taiwan.
3
School of Chinese Medicine, Graduate Institute of Chinese Medicine, Graduate Institute of Integrated Medicine, College of Chinese Medicine, Research Center for Chinese Medicine and Acupuncture, China Medical University, Taichung, 40447, Taiwan.
4
Departments of Medical Research, Obstetrics and Gynecology, Dermatology, and Urology, China Medical University Hospital, Taichung, 40447, Taiwan.
5
Department of Life Science, Tunghai University, Taichung, 40704, Taiwan.
6
Department of Surgery, Changhua Christian Hospital, 135 Nanhsiao Street, Changhua, 50094, Taiwan. d000103@cch.org.tw.
7
Vascular and Genomic Center, Changhua Christian Hospital, 135 Nanhsiao Street, Changhua, 50094, Taiwan. liu48111@gmail.com.
8
School of Chinese Medicine, Graduate Institute of Chinese Medicine, Graduate Institute of Integrated Medicine, College of Chinese Medicine, Research Center for Chinese Medicine and Acupuncture, China Medical University, Taichung, 40447, Taiwan. liu48111@gmail.com.
9
Department of Neurology, Changhua Christian Hospital, Changhua, 50094, Taiwan. liu48111@gmail.com.

Abstract

Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine neurodegenerative disease resulting from the misfolding and accumulation of a pathogenic protein, causing cerebellar dysfunction, and this disease currently has no effective treatments. Far-infrared radiation (FIR) has been found to protect the viability of SCA3 cells by preventing mutant ataxin-3 protein aggregation and promoting autophagy. However, this possible treatment still lacks in vivo evidence. This study assessed the effect of FIR therapy on SCA3 in vivo by using a mouse model over 28 weeks. Control mice carried a healthy wild-type ATXN3 allele that had a polyglutamine tract with 15 CAG repeats (15Q), whereas SCA3 transgenic mice possessed an allele with a pathological polyglutamine tract with expanded 84 CAG (84Q) repeats. The results showed that the 84Q SCA3 mice displayed impaired motor coordination, balance abilities, and gait performance, along with the associated loss of Purkinje cells in the cerebellum, compared with the normal 15Q controls; nevertheless, FIR treatment was sufficient to prevent those defects. FIR significantly improved performance in terms of maximal contact area, stride length, and base support in the forepaws, hindpaws, or both. Moreover, FIR treatment supported the survival of Purkinje cells in the cerebellum and promoted the autophagy, as reflected by the induction of autophagic markers, LC3II and Beclin-1, concomitant with the reduction of p62 and ataxin-3 accumulation in cerebellar Purkinje cells, which might partially contribute to the rescue mechanism. In summary, our results reveal that FIR confers therapeutic effects in an SCA3 transgenic animal model and therefore has considerable potential for future clinical use.

KEYWORDS:

Autophagy; Behavior; Far-infrared radiation; Purkinje cells; Spinocerebellar ataxia type 3; YAC transgenic mice

PMID:
29725949
DOI:
10.1007/s12311-018-0936-3
[Indexed for MEDLINE]

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