Pathology of myelinated axons in the PLP-deficient mouse model of spastic paraplegia type 2 revealed by volume imaging using focused ion beam-scanning electron microscopy.

Journal of Structural Biology
Anna M SteyerWiebke Möbius

Abstract

Advances in electron microscopy including improved imaging techniques and state-of-the-art detectors facilitate imaging of larger tissue volumes with electron microscopic resolution. In combination with genetic tools for the generation of mouse mutants this allows assessing the three-dimensional (3D) characteristics of pathological features in disease models. Here we revisited the axonal pathology in the central nervous system of a mouse model of spastic paraplegia type 2, the Plp-/Y mouse. Although PLP is a bona fide myelin protein, the major hallmark of the disease in both SPG2 patients and mouse models are axonal swellings comprising accumulations of numerous organelles including mitochondria, gradually leading to irreversible axonal loss. To assess the number and morphology of axonal mitochondria and the overall myelin preservation we evaluated two sample preparation techniques, chemical fixation or high-pressure freezing and freeze substitution, with respect to the objective of 3D visualization. Both methods allowed visualizing distribution and morphological details of axonal mitochondria. In Plp-/Y mice the number of mitochondria is 2-fold increased along the entire axonal length. Mitochondria are also found in the excess...Continue Reading

Citations

Sep 19, 2020·Histochemistry and Cell Biology·Jan Philipp SchneiderChristoph Wrede
Feb 25, 2021·The Journal of Eukaryotic Microbiology·Tomáš BílýHassan Hashimi

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Methods Mentioned

BETA
transmission electron microscopy
electron microscopy
imaging techniques

Software Mentioned

Bitplane
MIB
Imaris
Fiji
IMOD

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