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低酸的牙表现:翻译和临床进展

Elis J Lira Dos Santos¹, Fatma F Mohamed^1,2, Kaitrin Kramer^3,4

¹Division of Biosciences, College of Dentistry, The Ohio State University, Columbus, OH, 43210, United States.

|January 28, 2025

概括

低度症 (HPP) 是一种遗传性疾病,由于ALPL基因变异,影响骨和牙矿化. 酶替代疗法看起来很有前途,但基因疗法可能会为HPP患者的口腔健康提供进一步的改善.

科学领域:

生物化学生化学
遗传学遗传学是一个
代谢障碍代谢障碍代谢障碍

背景情况:

低度症 (HPP) 是一种遗传性代谢障碍,由ALPL基因中的功能丧失变异引起,该基因编码组织非特异性性酸酶 (TNAP).
TNAP对于生物矿物化至关重要,调节无机酸盐 (PPi) 水平,是骨和牙矿物化的抑制剂.
HPP在童年时表现为恶心病和生长缺陷,在成年时表现为骨质疏松症和骨折风险增加,显著影响口腔健康和牙气囊综合体.

研究的目的:

审查低酸盐 (HPP) 对牙和口腔健康的影响.
讨论目前对HPP对牙膜膜综合体的影响的理解,包括牙,牙,水泥和膜骨.
探索酶替代疗法 (ERT) 和新兴基因治疗方法在治疗HPP中的作用,特别是关于口腔健康结果.

主要方法:

关于HPP患者牙科护理的科学报告的文献综述.
对HPP动物模型的研究结果的分析,包括具有不同ALPL基因功能的敲除和敲进小鼠.
评估当前酶替代疗法 (ERT) 和临床前基因疗法研究的影响和局限性.

主要成果:

完全根植的牙的过早丧失是HPP的特征标志.
由于复杂的口腔健康问题,HPP需要涉及各种专家的多学科牙科护理.
虽然ERT改善了整体预后,但其对牙组织的具体影响尚不清楚,这凸显了需要进一步研究和替代疗法的需要.

结论:

HPP显著影响dentoalveolar复合体的四个硬组织,导致严重的口腔健康问题和过早的牙损失.
目前的酶替代疗法 (ERT) 在改善牙组织方面存在局限性,这促使人们对基因疗法的研究.
临床前基因疗法研究表明,它有可能改善低度症患者的口腔健康.

关键词:

牙科生物学牙科生物学与骨相关的/相关的疾病和疾病.遗传动物模型动物模型矩阵矿物化的矿化.骨质疏松症和恶心病的人.临床前研究临床前研究

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