Serial cryoFIB/SEM reveals cytoarchitectural disruptions in Leigh syndrome patient cells

Serial cryoFIB/SEM reveals cytoarchitectural disruptions in Leigh syndrome patient cells

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 optimi...

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التفاصيل البيبلوغرافية
المؤلفون الرئيسيون: Zhu, Y, Sun, D, Schertel, A, Ning, J, Fu, X, Gwo, PP, Watson, AM, Zanetti-Domingues, LC, Martin-Fernandez, ML, Freyberg, Z, Zhang, P
التنسيق: Journal article
اللغة:English
منشور في: Cell Press 2020
الوصف
الملخص: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.

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