Glutamate Dehydrogenase as a Promising Target for Hyperinsulinism
Hyperammonemia Syndrome Therapy

doi:10.2174/0929867328666210825105342
摘要

高胰岛素血症 - 高氨血症综合征（HHS）是一种罕见的疾病，其特征是复发性低血糖和血浆氨持续升高，可导致严重癫痫和永久性脑损伤。已经证明，谷氨酸脱氢酶 (GDH) 是线粒体基质中的一种酶，其功能突变是造成 HHS 的原因。因此，GDH已成为HHS小分子治疗干预的有希望的靶点。目前有几项药物化学研究针对GDH，然而，迄今为止，所报道的化合物均未进入临床试验。本文综述了GDH抑制剂的发现和开发进展，包括HHS的发病机制、潜在结合位点、筛选方法和研究模型。提供未来的治疗视角，为发现有效的GDH调节剂提供参考，并鼓励更多的研究，为药物开发提供更全面的指导。
关键词: 高胰岛素血症综合征（HHS），谷氨酸脱氢酶（GDH），筛选方法，酶抑制剂，药物发现，体外/体内模型。
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DOI https://dx.doi.org/10.2174/0929867328666210825105342	Print ISSN 0929-8673
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当代药物化学

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