Serial cryoFIB/SEM Reveals Cytoarchitectural Disruptions in Leigh Syndrome Patient Cells.
Serial cryoFIB/SEM Reveals Cytoarchitectural Disruptions in Leigh Syndrome Patient Cells.
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DOI：
10.1016/j.str.2020.10.003
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发表时间：
2021-01-07
期刊：
影响因子：
--
通讯作者：
Zhang P
中科院分区：
文献类型：
--
作者：
Zhu Y;Sun D;Schertel A;Ning J;Fu X;Gwo PP;Watson AM;Zanetti-Domingues LC;Martin-Fernandez ML;Freyberg Z;Zhang P
串行cryoFIB/SEM的进步提供了一个机会，研究大体积的近天然的，完全水合的冷冻细胞和组织的体素尺寸为10 nm及以下。我们通过开发和优化系列cryoFIB/SEM体积成像工作流程，探索了玻璃化冷冻人类患者细胞的病理表征能力。我们证明了Leigh综合征患者的原代成纤维细胞亚细胞结构的深刻破坏，该患者携带USMG 5蛋白的致病突变，导致线粒体能量产生受损。开发并优化了系列cryoFIB/SEM容积成像工作流程在天然条件下可视化整个细胞的3D结构揭示了原代LS患者成纤维细胞中细胞结构的破坏证明了致病组织临床表型分析的潜力系列cryoFIB/SEM为近天然条件下的细胞和组织结构分析提供了新的机会。Zhu等人开发并优化了一系列cryoFIB/SEM体积成像工作流程，用于在3D中可视化整个玻璃体细胞，并证明了其在原发性Leigh综合征患者细胞的临床和病理应用中的潜力。
The advancement of serial cryoFIB/SEM offers an opportunity to study large volumes of near-native, fully hydrated frozen cells and tissues at voxel sizes of 10 nm and below. We explored this capability for pathologic characterization of vitrified human patient cells by developing and optimizing a serial cryoFIB/SEM volume imaging workflow. We demonstrate profound disruption of subcellular architecture in primary fibroblasts from a Leigh syndrome patient harboring a disease-causing mutation in USMG5 protein responsible for impaired mitochondrial energy production. Developed and optimized a serial cryoFIB/SEM volume imaging workflow Visualized the 3D structure of an entire cell under native conditions Revealed a disruption of cellular structures in primary LS patient fibroblasts Demonstrated the potential for clinical phenotyping of pathogenic tissues Serial cryoFIB/SEM offers new opportunities for structural analysis of cells and tissues under near-native conditions. Zhu et al. developed and optimized a serial cryoFIB/SEM volume imaging workflow for visualization of entire vitreous cells in 3D, and demonstrated its potential for clinical and pathological applications with primary Leigh syndrome patient cells.
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影响因子：
3.6
作者：
McCormick EM;Zolkipli-Cunningham Z;Falk MJ
通讯作者：
Falk MJ
DOI：
10.1023/b:visi.0000029664.99615.94
发表时间：
2004-11-01
影响因子：
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作者：
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