| Literature DB >> 33096015 |
Yanan Zhu1, Dapeng Sun2, Andreas Schertel3, Jiying Ning2, Xiaofeng Fu2, Pam Pam Gwo4, Alan M Watson5, Laura C Zanetti-Domingues6, Marisa L Martin-Fernandez6, Zachary Freyberg7, Peijun Zhang8.
Abstract
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.Entities:
Keywords: CryoFIB/SEM; CryoSEM; Leigh syndrome; USMG5; block face; mitochondria disease; volume imaging
Year: 2020 PMID: 33096015 PMCID: PMC7802768 DOI: 10.1016/j.str.2020.10.003
Source DB: PubMed Journal: Structure ISSN: 0969-2126 Impact factor: 5.006