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  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. (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.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0001-69F2-8 Version Permalink: http://hdl.handle.net/21.11116/0000-0001-69F6-4
Genre: Journal Article

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Soliman, K., Author
Göttfert, F.1, Author              
Rosewich, H., Author
Thoms, S., Author
Gartner, J., Author
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1Department of NanoBiophotonics, MPI for Biophysical Chemistry, Max Planck Society, ou_578627              

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 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.

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Language(s): eng - English
 Dates: 2018-05-17
 Publication Status: Published online
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 Rev. Method: Peer
 Identifiers: DOI: 10.1038/s41598-018-24119-2
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Title: Scientific Reports
Source Genre: Journal
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Pages: 13 Volume / Issue: 8 Sequence Number: 7809 Start / End Page: - Identifier: -