Fission Impossible (?) – New Insights into Disorders of Peroxisome Dynamics
dc.contributor.author | Carmichael, RE | |
dc.contributor.author | Islinger, M | |
dc.contributor.author | Schrader, M | |
dc.date.accessioned | 2022-06-13T07:23:45Z | |
dc.date.issued | 2022-06-14 | |
dc.date.updated | 2022-06-10T16:49:40Z | |
dc.description.abstract | Peroxisomes are highly dynamic and responsive organelles, which can adjust their morphology, number, intracellular position, and metabolic functions according to cellular needs. Peroxisome multiplication in mammalian cells involves the concerted action of the membrane shaping protein PEX11β and division proteins such as the membrane adaptors FIS1 and MFF, which recruit the fission GTPase DRP1 to the peroxisomal membrane. The latter proteins are also involved in mitochondrial division. Patients with loss of DRP1, MFF or PEX11β function have been identified, showing abnormalities in peroxisomal (and, for the shared proteins, mitochondrial) dynamics as well as developmental and neurological defects, whereas metabolic functions of the organelles are often unaffected. Here, we provide a timely update on peroxisomal membrane dynamics with particular focus on peroxisome formation by membrane growth and division. We address the function of PEX11β in these processes, as well as the role of peroxisome-ER contacts in lipid transfer for peroxisomal membrane expansion. Furthermore, we summarize the clinical phenotypes and pathophysiology of patients with defects in the key division proteins DRP1, MFF, and PEX11β as well as in the peroxisome-ER tether ACBD5. Potential therapeutic strategies for these rare disorders with limited treatment options are discussed | en_GB |
dc.description.sponsorship | Biotechnology & Biological Sciences Research Council (BBSRC) | en_GB |
dc.identifier.citation | Vol. 11 (12), article 1922 | en_GB |
dc.identifier.doi | 10.3390/cells11121922 | |
dc.identifier.grantnumber | BB/T002255/1 | en_GB |
dc.identifier.grantnumber | BB/R016844/1 | en_GB |
dc.identifier.uri | http://hdl.handle.net/10871/129917 | |
dc.identifier | ORCID: 0000-0003-2146-0535 (Schrader, Michael) | |
dc.language.iso | en | en_GB |
dc.publisher | MDPI | en_GB |
dc.rights | © 2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). | en_GB |
dc.subject | peroxisomes | en_GB |
dc.subject | mitochondria | en_GB |
dc.subject | organelle dynamics | en_GB |
dc.subject | division defects | en_GB |
dc.subject | dynamin-related protein 1 | en_GB |
dc.subject | mitochondrial fission factor | en_GB |
dc.subject | PEX11β | en_GB |
dc.subject | FIS1 | en_GB |
dc.subject | ACBD5 | en_GB |
dc.subject | membrane fission | en_GB |
dc.title | Fission Impossible (?) – New Insights into Disorders of Peroxisome Dynamics | en_GB |
dc.type | Article | en_GB |
dc.date.available | 2022-06-13T07:23:45Z | |
dc.identifier.issn | 2073-4409 | |
dc.description | This is the final version. Available on open access from MDPI via the DOI in this record | en_GB |
dc.description | Data Availability Statement: All datasets generated for this study are included in the article. | en_GB |
dc.identifier.journal | Cells | en_GB |
dc.rights.uri | https://creativecommons.org/licenses/by/4.0/ | en_GB |
dcterms.dateAccepted | 2022-06-10 | |
rioxxterms.version | VoR | en_GB |
rioxxterms.licenseref.startdate | 2022-06-10 | |
rioxxterms.type | Journal Article/Review | en_GB |
refterms.dateFCD | 2022-06-10T16:49:43Z | |
refterms.versionFCD | AM | |
refterms.dateFOA | 2022-06-28T14:15:56Z | |
refterms.panel | A | en_GB |
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Except where otherwise noted, this item's licence is described as © 2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).