Super-resolution imaging reveals the sub-diffraction phenotype of Zellweger 
Syndrome ghosts and wild-type peroxisomes.

Soliman, K.; Göttfert, F.; Rosewich, H.; Thoms, S.; Gartner, J.

doi:10.1038/s41598-018-24119-2

Item

ITEM ACTIONSEXPORT

Add to Basket

Please note that a newer version of this item is available:
https://pure.mpg.de/pubman/item/item_2596680_6

DetailsSummary

Released

Journal Article

Super-resolution imaging reveals the sub-diffraction phenotype of Zellweger Syndrome ghosts and wild-type peroxisomes.

MPS-Authors

/persons/resource/persons96560

Göttfert, F.
Department of NanoBiophotonics, MPI for Biophysical Chemistry, Max Planck Society;

External Resource

No external resources are shared

Fulltext (restricted access)

There are currently no full texts shared for your IP range.

Fulltext (public)

2596680.pdf
(Publisher version), 3MB

Supplementary Material (public)

2596680_Suppl.pdf
(Supplementary material), 3MB

Citation

Soliman, K., Göttfert, F., Rosewich, H., Thoms, S., & Gartner, J. (2018). Super-resolution imaging reveals the sub-diffraction phenotype of Zellweger Syndrome ghosts and wild-type peroxisomes. Scientific Reports, 8: 7809. doi:10.1038/s41598-018-24119-2.

Cite as: https://hdl.handle.net/21.11116/0000-0001-69F2-8

Abstract

Peroxisomes are ubiquitous cell organelles involved in many metabolic and signaling functions. Their assembly requires peroxins, encoded by PEX genes. Mutations in PEX genes are the cause of Zellweger Syndrome spectrum (ZSS), a heterogeneous group of peroxisomal biogenesis disorders (PBD). The size and morphological features of peroxisomes are below the diffraction limit of light, which makes them attractive for super-resolution imaging. We applied Stimulated Emission Depletion (STED) microscopy to study the morphology of human peroxisomes and peroxisomal protein localization in human controls and ZSS patients. We defined the peroxisome morphology in healthy skin fibroblasts and the sub-diffraction phenotype of residual peroxisomal structures ('ghosts') in ZSS patients that revealed a relation between mutation severity and clinical phenotype. Further, we investigated the 70 kDa peroxisomal membrane protein (PMP70) abundance in relationship to the ZSS sub-diffraction phenotype. This work improves the morphological definition of peroxisomes. It expands current knowledge about peroxisome biogenesis and ZSS pathoethiology to the sub-diffraction phenotype including key peroxins and the characteristics of ghost peroxisomes.