{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Verhoeven N"],"funding":["University of Maryland, Baltimore County","National Institutes of Health","National Institute of General Medical Sciences","NIGMS NIH HHS"],"pagination":["40-50.e5"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11706706"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["60(1)"],"pubmed_abstract":["We report that the outer mitochondrial membrane (OMM)-associated E3 Ub ligase MARCH5 is vital for generating mitochondria-derived pre-peroxisomes. In human immortalized cells, MARCH5 knockout leads to the accumulation of immature peroxisomes, reduced fatty-acid-induced peroxisomal biogenesis, and abnormal peroxisome biogenesis in MARCH5/Pex14 and MARCH5/Pex3 dko cells. Upon fatty-acid-induced peroxisomal biogenesis, MARCH5 redistributes to peroxisomes, and ubiquitination activity-deficient mutants of MARCH5 accumulate on peroxisomes containing high levels of the OMM protein Tom20 (mitochondria-derived pre-peroxisomes). Similarly, depletion of peroxisome biogenesis factor Pex14 leads to the accumulation of MARCH5- and Tom20-positive pre-peroxisomes, whereas no peroxisomes are detected in MARCH5/Pex14 dko cells. Inconsistent with MARCH5 merely acting as a quality factor, mitochondrial decline is not evident in tested models. Furthermore, reduced expression of peroxisomal proteins is detected in MARCH5<sup>-/-</sup> cells, whereas some of these proteins are stabilized in peroxisome biogenesis deficiency models lacking MARCH5 expression. Thus, MARCH5 is central for mitochondria-dependent peroxisome biogenesis."],"journal":["Developmental cell"],"pubmed_title":["Outer mitochondrial membrane E3 Ub ligase MARCH5 controls de novo peroxisome biogenesis."],"pmcid":["PMC11706706"],"funding_grant_id":["R01GM129584","R01 GM129584"],"pubmed_authors":["Cartier E","Neutzner A","Boyman L","Bippes CC","Karbowski M","Verhoeven N","Oshima Y"],"additional_accession":[]},"is_claimable":false,"name":"Outer mitochondrial membrane E3 Ub ligase MARCH5 controls de novo peroxisome biogenesis.","description":"We report that the outer mitochondrial membrane (OMM)-associated E3 Ub ligase MARCH5 is vital for generating mitochondria-derived pre-peroxisomes. In human immortalized cells, MARCH5 knockout leads to the accumulation of immature peroxisomes, reduced fatty-acid-induced peroxisomal biogenesis, and abnormal peroxisome biogenesis in MARCH5/Pex14 and MARCH5/Pex3 dko cells. Upon fatty-acid-induced peroxisomal biogenesis, MARCH5 redistributes to peroxisomes, and ubiquitination activity-deficient mutants of MARCH5 accumulate on peroxisomes containing high levels of the OMM protein Tom20 (mitochondria-derived pre-peroxisomes). Similarly, depletion of peroxisome biogenesis factor Pex14 leads to the accumulation of MARCH5- and Tom20-positive pre-peroxisomes, whereas no peroxisomes are detected in MARCH5/Pex14 dko cells. Inconsistent with MARCH5 merely acting as a quality factor, mitochondrial decline is not evident in tested models. Furthermore, reduced expression of peroxisomal proteins is detected in MARCH5<sup>-/-</sup> cells, whereas some of these proteins are stabilized in peroxisome biogenesis deficiency models lacking MARCH5 expression. Thus, MARCH5 is central for mitochondria-dependent peroxisome biogenesis.","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025 Jan","modification":"2026-06-06T20:04:13.175Z","creation":"2026-06-04T03:14:56.112Z"},"accession":"S-EPMC11706706","cross_references":{"pubmed":["39423819"],"doi":["10.1016/j.devcel.2024.09.010"]}}