Progressive rod bipolar cell loss, ubiquitin accumulation, and subretinal drusenoid deposits in Cln3Δex7/8 mice: New insights into juvenile neuronal ceroid lipofuscinosis pathogenesis.
第一作者:
Tommi,Torsti
第一单位:
School of Pharmacy, University of Eastern Finland, Kuopio, Finland. Electronic address: tommi.torsti@uef.fi.
作者:
医学主题词
动物(Animals);神经元蜡样质脂褐质沉积病(Neuronal Ceroid-Lipofuscinoses);视网膜电描记术(Electroretinography);小鼠(Mice);体层摄影术, 光学相干(Tomography, Optical Coherence);疾病模型, 动物(Disease Models, Animal);视网膜双极细胞(Retinal Bipolar Cells);分子伴侣(Molecular Chaperones);视网膜小疣(Retinal Drusen);膜糖蛋白类(Membrane Glycoproteins);泛素(Ubiquitin);视网膜视杆光感受器细胞(Retinal Rod Photoreceptor Cells);小鼠, 近交C57BL(Mice, Inbred C57BL);疾病恶化(Disease Progression)
DOI
10.1016/j.exer.2025.110766
PMID
41274622
发布时间
2026-01-08
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Experimental eye research
110766页
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