Blanco-Hernán Pedro, Aguado Lorena, Asensio María José, Gómez-Soria Ana, de la Villa Pedro, Casarejos María José, Mansilla Alicia
Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), 28034 Madrid, Spain.
Servicio de Neurobiología-Investigación, Hospital Ramón y Cajal, 28034 Madrid, Spain.
Int J Mol Sci. 2025 Jun 10;26(12):5532. doi: 10.3390/ijms26125532.
Retinal dysfunction is emerging as a potential early marker of neurodegenerative diseases. Within the retina, the dopaminergic circuit, comprising dopaminergic amacrine cells, dopamine synthesis and turnover, and dopamine receptor signalling, is essential for visual processing, particularly colour contrast perception. Disruption of this circuit may underline early retinal alterations observed in Huntington's disease (HD) and Alzheimer's disease (AD). In this study, we systematically analysed retinal dopaminergic dysfunction in murine models of HD (genetic origin) and AD (sporadic), across different disease stages. We assessed dopamine levels, turnover, tyrosine hydroxylase expression, D1 and D2 receptor gene expression, and neurotransmitter balance. HD mice showed early and marked alterations: reduced dopamine content, decreased tyrosine hydroxylase, increased turnover, and downregulation of D1 receptor expression-all preceding motor symptoms and detectable brain pathology. In contrast, AD mice showed only mild changes at later stages; however, clinical evidence suggests that similar dysfunction may occur earlier in human AD. These findings position retinal dopaminergic disruption as a potential early biomarker in HD and possibly in AD. While the current study relies on invasive techniques in animal models, it lays the groundwork for non-invasive retinal assessments, such as electroretinography or optical coherence tomography, as promising tools for early diagnosis and disease monitoring in neurodegeneration.
视网膜功能障碍正逐渐成为神经退行性疾病的一种潜在早期标志物。在视网膜内,多巴胺能回路,包括多巴胺能无长突细胞、多巴胺合成与周转以及多巴胺受体信号传导,对于视觉处理,尤其是颜色对比度感知至关重要。该回路的破坏可能是亨廷顿舞蹈病(HD)和阿尔茨海默病(AD)中观察到的早期视网膜改变的潜在原因。在本研究中,我们系统地分析了HD(遗传起源)和AD(散发性)小鼠模型在不同疾病阶段的视网膜多巴胺能功能障碍。我们评估了多巴胺水平、周转、酪氨酸羟化酶表达、D1和D2受体基因表达以及神经递质平衡。HD小鼠表现出早期且明显的改变:多巴胺含量降低、酪氨酸羟化酶减少、周转增加以及D1受体表达下调——所有这些都先于运动症状和可检测到的脑部病理变化。相比之下,AD小鼠仅在后期表现出轻微变化;然而,临床证据表明,类似的功能障碍可能在人类AD中更早出现。这些发现表明视网膜多巴胺能破坏是HD以及可能是AD中的一种潜在早期生物标志物。虽然目前的研究依赖于动物模型中的侵入性技术,但它为非侵入性视网膜评估,如视网膜电图或光学相干断层扫描,奠定了基础,这些技术有望成为神经退行性疾病早期诊断和疾病监测的工具。