Mitochondrial transplantation restores mitochondrial content and function in SSBP1-related mitochondrial DNA depletion syndrome.

Dohee Kim, Seo-Eun Lee, JuHyuen Cha, Jun Ho Lee, Young Cheol Kang, Sang-Yeon Lee

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In one paragraph

Article in BMB reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

6 authors.

Dohee KimDepartment of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea.

Seo-Eun LeePaean Biotechnology, Inc., Seoul 04552, Korea.

JuHyuen ChaDepartment of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea.

Jun Ho LeeDepartment of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea.

Young Cheol KangPaean Biotechnology, Inc., Seoul 04552, Korea.

Sang-Yeon LeeDepartment of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080; Sensory Organ Research Institute, Seoul National University Medical Research Center, Seoul 03080, Korea.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

This study examined therapeutic potential of mitochondrial transplantation using PN-101, a mitochondria preparation derived from human umbilical cord mesenchymal stem cells (UCMSCs), to address SSBP1-related mitochondrial DNA (mtDNA) depletion syndrome. Patient-derived fibroblasts harboring a heterozygous SSBP1 mutation (c.272G＞A:p.Arg91Gln) were treated with PN-101. Its successful uptake and integration into these cells were confirmed. Subsequent analyses revealed that PN-101 treatment significantly increased mtDNA copy numbers in a time- and dose-dependent manner, elevated the expression of key oxidative phosphorylation proteins, and enhanced overall mitochondrial bioenergetics. Taken together, these results provide strong evidence that mitochondrial transplantation holds promise as a therapeutic strategy for primary mitochondrial diseases, including those involving SSBP1 mutations. [BMB Reports 2026; 59(4): 227-234].

Indexed as

DNA-Binding ProteinsDNA, MitochondrialMitochondriaMitochondrial DiseasesCells, CulturedFibroblastsHumansMesenchymal Stem CellsMitochondrial ProteinsMutationOxidative PhosphorylationDNA-Binding ProteinsDNA, MitochondrialMitochondrial ProteinsNABP2 protein, human

Identifiers

PMID41261791

PMCPMC13126069

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LicenceCC BY-NC

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.