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Ion-abrasion scanning electron microscopy reveals distorted liver mitochondrial morphology in murine methylmalonic acidemia.
- Source :
-
Journal of structural biology [J Struct Biol] 2010 Aug; Vol. 171 (2), pp. 125-32. Date of Electronic Publication: 2010 Apr 23. - Publication Year :
- 2010
-
Abstract
- Methylmalonic acidemia is a lethal inborn error of metabolism that causes mitochondrial impairment, multi-organ dysfunction and a shortened lifespan. Previous transmission electron microscope studies of thin sections from normal (Mut(+/+)) and diseased (Mut(-/-)) tissue found that the mitochondria appear to occupy a progressively larger volume of mutant cells with age, becoming megamitochondria. To assess changes in shape and volume of mitochondria resulting from the mutation, we carried out ion-abrasion scanning electron microscopy (IA-SEM), a method for 3D imaging that involves the iterative use of a focused gallium ion beam to abrade the surface of the specimen, and a scanning electron beam to image the newly exposed surface. Using IA-SEM, we show that mitochondria are more convoluted and have a broader distribution of sizes in the mutant tissue. Compared to normal cells, mitochondria from mutant cells have a larger surface-area-to-volume ratio, which can be attributed to their convoluted shape and not to their elongation or reduced volume. The 3D imaging approach and image analysis described here could therefore be useful as a diagnostic tool for the evaluation of disease progression in aberrant cells at resolutions higher than that currently achieved using confocal light microscopy.<br /> (Published by Elsevier Inc.)
- Subjects :
- Amino Acid Metabolism, Inborn Errors pathology
Animals
Blotting, Western
Mice
Microscopy, Confocal
Mitochondria, Liver pathology
Models, Biological
Oxidative Phosphorylation
Amino Acid Metabolism, Inborn Errors metabolism
Methylmalonic Acid metabolism
Microscopy, Electron, Scanning
Mitochondria, Liver ultrastructure
Subjects
Details
- Language :
- English
- ISSN :
- 1095-8657
- Volume :
- 171
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Journal of structural biology
- Publication Type :
- Academic Journal
- Accession number :
- 20399866
- Full Text :
- https://doi.org/10.1016/j.jsb.2010.04.005