Ameliorative Effects of Citrus Unshiu Peel Extract in Cellular and Mouse Models of Pelizaeus–Merzbacher Disease
- 1Department of Precision Medicine and Drug Discovery, National Research Institute for Child Health and Development, Tokyo 157-8535, Japan
- 2Tsumura Advanced Technology Research Laboratories, Tsumura & Co., Ibaraki 300-1192, Japan
- 3Laboratory of Molecular Neuroscience and Neurology, Tokyo University of Pharmacy and Life Sciences, Tokyo 192-0392, Japan
Abstract
Hypomyelinating leukodystrophy 1 (HLD1), also known as Pelizaeus–Merzbacher disease (PMD), is a severe X-linked hereditary hypomyelinating/demyelinating disorder caused primarily by genomic duplications or multiplications that lead to the increased expression of proteolipid protein 1 (PLP1). Aberrant PLP1 expression impairs oligodendrocyte differentiation and disrupts myelin formation in the central nervous system (CNS). Despite decades of research, effective disease-modifying therapies remain unavailable. In the present study, we investigated the potential of Chinpi, the Citrus Unshiu Peel extract commonly utilized in traditional herbal medicine and reported to protect cells from various cellular stresses, using both in vitro cellular and in vivo animal models of PMD. Using an FBD-102b oligodendrocyte precursor model cell line engineered to overexpress PLP1, we demonstrated that treatment with Chinpi significantly ameliorates critical defects in morphological differentiation and successfully restores the expression of major myelin proteins. Furthermore, oral administration of Chinpi to PLP1 transgenic mice improves myelin protein expression in the brain and significantly alleviates progressive motor incoordination, as assessed by behavioral tests. Taken together, these findings suggest that Chinpi exerts protective effects against PLP1-induced morphological differentiation defects and subsequent reductions in myelin protein expression, highlighting its potential as a recovery approach for PMD.
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BibTeX
@article{yamamoto2026ameliorative,
title = {{Ameliorative Effects of Citrus Unshiu Peel Extract in Cellular and Mouse Models of Pelizaeus–Merzbacher Disease}},
author = {Miyu Yamamoto and Hideya Sakaguchi and Moeri Yagi and Yuta Muraki and Akinori Nishi and Hideji Yako and Junji Yamauchi and Yuki Miyamoto},
journal = {International Journal of Molecular Sciences},
year = {2026},
month = sep,
doi = {10.3390/ijms27198732},
url = {https://www.mdpi.com/1422-0067/27/19/8732},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Ameliorative Effects of Citrus Unshiu Peel Extract in Cellular and Mouse Models of Pelizaeus–Merzbacher Disease
AU - Miyu Yamamoto
AU - Hideya Sakaguchi
AU - Moeri Yagi
AU - Yuta Muraki
AU - Akinori Nishi
AU - Hideji Yako
AU - Junji Yamauchi
AU - Yuki Miyamoto
PY - 2026
DA - 2026/09/30
JO - International Journal of Molecular Sciences
DO - 10.3390/ijms27198732
UR - https://www.mdpi.com/1422-0067/27/19/8732
AB - Hypomyelinating leukodystrophy 1 (HLD1), also known as Pelizaeus–Merzbacher disease (PMD), is a severe X-linked hereditary hypomyelinating/demyelinating disorder caused primarily by genomic duplications or multiplications that lead to the increased expression of proteolipid protein 1 (PLP1). Aberrant PLP1 expression impairs oligodendrocyte differentiation and disrupts myelin formation in the central nervous system (CNS). Despite decades of research, effective disease-modifying therapies remain unavailable. In the present study, we investigated the potential of Chinpi, the Citrus Unshiu Peel extract commonly utilized in traditional herbal medicine and reported to protect cells from various cellular stresses, using both in vitro cellular and in vivo animal models of PMD. Using an FBD-102b oligodendrocyte precursor model cell line engineered to overexpress PLP1, we demonstrated that treatment with Chinpi significantly ameliorates critical defects in morphological differentiation and successfully restores the expression of major myelin proteins. Furthermore, oral administration of Chinpi to PLP1 transgenic mice improves myelin protein expression in the brain and significantly alleviates progressive motor incoordination, as assessed by behavioral tests. Taken together, these findings suggest that Chinpi exerts protective effects against PLP1-induced morphological differentiation defects and subsequent reductions in myelin protein expression, highlighting its potential as a recovery approach for PMD.
SN - 1422-0067
ER -