OXPHOS upregulation to preserve vision in Leber's Hereditary Optic Neuropathy

OXPHOS 上调可保护莱伯遗传性视神经病患者的视力

• 批准号: nhmrc : 1061472
• 负责人: A/Pr Ian Trounce
• 金额: $ 33.13万
• 依托单位: Centre for Eye Research Australia Ltd
• 依托单位国家: 澳大利亚
• 项目类别: Project Grants
• 财政年份: 2014
• 资助国家: 澳大利亚
• 起止时间: 2014-01-01 至 2016-12-31
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/oxphos-upregulation-preserve-optic-neuropathy/454902
• 关键词: OXPHOS; upregulation; preserve; vision; Leber

Leber's Hereditary Optic Neuropathy (LHON) is a devastating blinding disease that preferentially affects young men. Sufferers have normal vision until teenage years or their twenties when a rapid loss of vision occurs that results in permanent blindness. It is caused by genetic changes in the mitochondrial DNA that we inherit from our mothers. The mitochondria are the cells' energy generators. We aim to use molecules similar to female hormones to boost energy as a new treatment to preserve vision in at-risk LHON individuals.

Leber遗传性视神经病变（LHON）是一种毁灭性的致盲疾病，优先影响年轻男性。患者的视力正常，直到十几岁或二十多岁时，视力迅速丧失，导致永久性失明。它是由我们从母亲那里继承的线粒体DNA的遗传变化引起的。线粒体是细胞的能量发生器。我们的目标是使用类似于女性激素的分子来提高能量，作为一种新的治疗方法来保护处于风险中的LHON个体的视力。