4.7 Article

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

期刊

STRUCTURE
卷 29, 期 1, 页码 82-+

出版社

CELL PRESS
DOI: 10.1016/j.str.2020.10.003

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资金

  1. Department of Defense [PR141292, PR192466]
  2. NIH [AI150481]
  3. UK Wellcome Trust Investigator Award [206422/Z/17/Z]
  4. UK Biotechnology and Biological Sciences Research Council [BB/S003339/1]
  5. BBSRC [BB/S003339/1] Funding Source: UKRI

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The advancement of serial cryoFIB/SEM technology allows for the study of large volumes of near-native, fully hydrated frozen cells and tissues at voxel sizes of 10 nm and below. This capability was utilized to characterize the 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.
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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