書誌事項
- タイトル別名
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- Prenatal enzyme replacement therapy restores delayed calcification in the maxillofacial region of mice with severe infantile hypophosphatemia.
抄録
<p>Hypophosphatasia (HPP) is a congenital disease caused by a deficiency of tissue-nonspecific alkaline phosphatase (TNALP) gene. The pathogenesis of HPP varies, ranging from severe cases resulting in no fetal bone calcification to relatively mild cases. In recent years, the establishment of enzyme supplementation as a treatment method has prolonged survival in patients. However, the effects of enzyme therapy on the jawbone and periodontal tissues have not yet been studied in detail. Therefore, in this study, we investigated the therapeutic effects of enzyme replacement therapy on jawbone in mice. Recombinant TNALP was administered to mothers before birth and newborns immediately after birth, with the effect of treatment being evaluated. The treated HPP mice had improved mandibular bone quality and tooth quality (root length of mandibular first molar, formation of cementum), as well as improved periodontal ligament structure. Furthermore, prenatal treatment had an additional therapeutic effect on the degree of mandible and enamel calcification. These results suggest that enzyme replacement therapy is effective for the treatment of HPP, specifically in the maxillofacial region, and that early initiation of treatment may have additional beneficial therapeutic effects.</p>
収録刊行物
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- 日本薬理学会年会要旨集
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日本薬理学会年会要旨集 97 (0), 3-B-P-050-, 2023
公益社団法人 日本薬理学会
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キーワード
詳細情報 詳細情報について
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- CRID
- 1390580217715747712
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- ISSN
- 24354953
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- 本文言語コード
- ja
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- データソース種別
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- JaLC
- Crossref
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- 抄録ライセンスフラグ
- 使用不可