Serial CryoFIB/SEM Reveals Cytoarchitectural Disruptions in Leigh Syndrome Patient Cells

Overview

Journal Structure

Publisher Cell Press

Specialties Biochemistry
Biotechnology
Cell Biology
Molecular Biology

Date 2020 Oct 23

PMID 33096015

Citations 15

Authors

Yanan Zhu

Dapeng Sun

Andreas Schertel

Jiying Ning

Xiaofeng Fu

Pam Pam Gwo

Alan M Watson

Laura C Zanetti-Domingues

Marisa L Martin-Fernandez

Zachary Freyberg

Peijun Zhang

Affiliations

Soon will be listed here.

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.

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