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

ABSTRACT:

SUBMITTER: Peijun Zhang

PROVIDER: EMPIAR-10515 | biostudies-other |

REPOSITORIES: biostudies-other

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Serial cryoFIB/SEM Reveals Cytoarchitectural Disruptions in Leigh Syndrome Patient Cells.

Zhu Yanan Y Sun Dapeng D Schertel Andreas A Ning Jiying J Fu Xiaofeng X Gwo Pam Pam PP Watson Alan M AM Zanetti-Domingues Laura C LC Martin-Fernandez Marisa L ML Freyberg Zachary Z Zhang Peijun P

Structure (London, England : 1993) 20201022 1

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 US ...[more]

PMID: 33096015

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