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Brain N-aetylaspartate is elevated in Pelizaeus-Merzbacher disease with PLP1 duplication
https://repo.qst.go.jp/records/44015
https://repo.qst.go.jp/records/4401511f757f0-29a7-47c0-ba9f-4dccfa8c1426
| Item type | 学術雑誌論文 / Journal Article(1) | |||||
|---|---|---|---|---|---|---|
| 公開日 | 2006-03-15 | |||||
| タイトル | ||||||
| タイトル | Brain N-aetylaspartate is elevated in Pelizaeus-Merzbacher disease with PLP1 duplication | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | journal article | |||||
| アクセス権 | ||||||
| アクセス権 | metadata only access | |||||
| アクセス権URI | http://purl.org/coar/access_right/c_14cb | |||||
| 著者 |
Takanashi, Junichi
× Takanashi, Junichi× Ikehira, Hiroo× Tanada, Shuji× Yoshitome, Eiji× et.al× 高梨 潤一× 池平 博夫× 棚田 修二× 吉留 英二 |
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| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Obfective:To assess alterations in brain metabolites of patients with Pelizaeus-Merzbacher disease (PMD) with the proteolipid protein gene 1 (PLP1) duplications using quantitative proton MRS. Methods:Five unrelated male Japanese patients with PMD with PLP1 duplications were analyzed using automated proton brain examination with the point resolved spectroscopy technique (repetition and echo time of 5,000 and 30 msec). Localized spectra in the posterior portion of the centrum semiovale were acquired, and absolute metabolite concentrations were calculated using the LCModel. Results:Absolute concentrations of N-acetylaspartate (NAA),creatine (Cr),and myoinositol(MI) were increased by 16%(p<0.01),and 43%(p<0.001),and 31%(p<0.01)in patients with PMD as compared with age-matched controls,There was no statistical difference in choline concentration. Conclusion:The increased concentration of NAA,which could not be detected by previous relative quantitation methods, suggests two possibilities: axonal involvement secondary to dysmyelination, or increased cell population of oligoddendrocyte progenitors. Elevated Cr and MI concentrations may reflect the reactive astrocytic gliosis. Our study thus emphasizes the importance of absolute quantitation of metabolites to investigate the disease mechanism of the dysmyelinating disorders of the CNS. |
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| 書誌情報 |
Neurology 巻 58, 号 2, p. 237-241, 発行日 2002-01 |
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| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 0028-3878 | |||||
