Affinage

SSBP1

Single-stranded DNA-binding protein, mitochondrial · UniProt Q04837

Length
148 aa
Mass
17.3 kDa
Annotated
2026-06-10
50 papers in source corpus 18 papers cited in narrative 18 extracted findings
Cross-family judge vs UniProt: tie faithfulness: 5/5 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SSBP1 (mtSSB) is the mitochondrial single-stranded DNA-binding protein that serves as a core component of the mtDNA replisome, assembling into tetramers and stimulating both DNA polymerase γ and the Twinkle helicase through distinct surface elements removed from its ssDNA-binding groove to drive mtDNA replication (PMID:21953457). Disease-associated missense substitutions in conserved arginines of the basic ssDNA-binding patch (R38Q, R107Q, S141N) act through multiple biophysical routes—destabilizing dimer/tetramer assembly and distorting the DNA-binding region (R38Q) or, without disrupting tetramers, reducing ssDNA compaction and accelerating dissociation (R107Q)—producing dominant-negative loss of replication competence, mtDNA depletion, and the optic-atrophy/multisystem mitochondrial phenotypes seen in patients and zebrafish models (PMID:31550237, PMID:31550240, PMID:31298765, PMID:38742632). SSBP1 haploinsufficiency from a heterozygous start-loss allele likewise lowers protein levels and causes tissue-specific mtDNA depletion and deletions, compounding a separate mitochondrial translation defect (PMID:29182774). Beyond replication, SSBP1 localizes to mitochondrial RNA granules and participates in the GRSF1–mtRNA degradosome pathway, where its depletion causes mtRNA processing defects and accumulation of dsRNA and RNA:DNA hybrids (PMID:30715486), and it shields ssDNA from spurious base-excision processing by directly binding alkyladenine DNA glycosylase (AAG) and inhibiting its activity on single-stranded substrates (PMID:23290262, PMID:22153281). Under proteotoxic stress, mitochondrial SSBP1 translocates to the nucleus via the ANT-VDAC1 pore and direct HSF1 interaction, where the HSF1–SSBP1 complex recruits the chromatin remodeller BRG1 to chaperone gene promoters to boost their induction and support cell survival (PMID:25762445).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2007 Medium

    Established in vivo that the SSBP1 ortholog is required for mtDNA replication and the downstream cellular processes that depend on it, framing it as an essential maintenance factor rather than a dispensable accessory.

    Evidence Generational RNAi depletion of C. elegans par2.1/mtssb-1 with mtDNA copy number, cytology, and transcriptome readouts

    PMID:17900564

    Open questions at the time
    • Ortholog-based; does not resolve the molecular mechanism by which mtSSB acts at the fork
    • Germline/proliferation phenotypes are indirect consequences of mtDNA loss
  2. 2011 High

    Resolved how mtSSB drives replication: it uses distinct surface elements to independently stimulate polymerase γ and the helicase, answering whether its replisome roles are separable.

    Evidence In vitro polymerase and unwinding assays with surface-residue mutants plus mtDNA copy number in Drosophila S2 cells

    PMID:21953457

    Open questions at the time
    • Did not define the structural basis of stimulation at atomic resolution
    • Helicase identity stimulated in cells inferred from in vitro work
  3. 2011 Medium

    Showed that mtSSB protects replicating ssDNA from break-generating repair chemistry, recasting it as a guardian against aberrant base-excision intermediates.

    Evidence In vitro base-excision/repair enzyme assays (UNG1, ABH1, NEIL1, APE1) on ssDNA vs dsDNA with purified mtSSB

    PMID:22153281

    Open questions at the time
    • In vitro only; cellular relevance of the inhibition not tested
    • No structural map of the inhibitory interface
  4. 2013 Medium

    Identified a direct partner for the ssDNA-protection role, showing mtSSB binds AAG and selectively blocks its glycosylase activity on single-stranded substrates.

    Evidence Mitochondrial immunofluorescence, pulldown binding assay, and in vitro glycosylase assays on ssDNA/dsDNA

    PMID:23290262

    Open questions at the time
    • Proposed UNG1 recruitment motif not directly demonstrated
    • Single lab; physiological significance during repair in cells not established
  5. 2015 High

    Revealed a non-canonical nuclear function: under proteotoxic stress mtSSB exits mitochondria to co-activate the HSF1 chaperone program, linking mitochondrial DNA biology to the cytoprotective stress response.

    Evidence Co-IP, ChIP, live-cell translocation imaging, and loss-of-function survival/membrane-potential assays

    PMID:25762445

    Open questions at the time
    • Mechanism releasing SSBP1 from the nucleoid pool unclear
    • Relationship between nuclear and replisome pools not quantified
  6. 2019 Medium

    Extended SSBP1 function from DNA to RNA, placing it in mitochondrial RNA granules and the GRSF1 degradosome pathway for processing G-quadruplex-prone mtRNAs.

    Evidence Immunofluorescence colocalization, siRNA depletion, RNA processing, dsRNA and RNA:DNA hybrid detection

    PMID:30715486

    Open questions at the time
    • Whether RNA-granule role is separable from replisome role unresolved
    • Direct RNA-binding versus indirect recruitment not distinguished
  7. 2019 Medium

    Connected SSBP1 to inherited optic-atrophy/retinal disease by showing basic-patch arginine variants act dominant-negatively to impair ssDNA binding.

    Evidence In silico structural analysis and zebrafish antisense knockdown with wild-type/mutant mRNA rescue scoring retinal ganglion cell differentiation

    PMID:31298765

    Open questions at the time
    • Structural interpretation computational
    • Biophysical mechanism of each variant not measured directly here
  8. 2019 Medium

    Documented that SSBP1 haploinsufficiency compounds an independent mitochondrial translation defect, demonstrating dosage sensitivity and tissue-specific consequences.

    Evidence Exome sequencing of a family, SSBP1 western blot, and mtDNA copy number/deletion analysis in skeletal muscle

    PMID:29182774

    Open questions at the time
    • Single family
    • Tissue specificity of phenotype mechanistically unexplained
  9. 2020 High

    Provided structural and patient-cell mechanism for disease variants, showing R38Q/R107Q disrupt dimer interactions and the DNA-binding region to cause mtDNA depletion.

    Evidence Crystal structure, size-exclusion oligomeric analysis, patient fibroblast and in vitro replication assays, and zebrafish rescue across two independent groups

    PMID:31550237 PMID:31550240

    Open questions at the time
    • Dynamics of ssDNA engagement not captured by static crystal structure
    • How partial oligomer defects translate to depletion thresholds unclear
  10. 2024 High

    Refined the variant mechanism at single-molecule resolution, showing R107Q does not destabilize tetramers but impairs ssDNA compaction and accelerates dissociation, distinguishing binding kinetics from assembly defects.

    Evidence Single-molecule manipulation, compaction and dissociation kinetics, real-time competition binding, and molecular modeling

    PMID:38742632

    Open questions at the time
    • Reconciliation with earlier tetramer-destabilization reports for the same residue context not fully resolved
    • In vivo correlation of kinetic deficits to depletion severity untested
  11. 2024 Medium

    Proposed that mtSSB positively regulates Twinkle by outcompeting its zinc-binding domain for DNA, relieving an auto-inhibitory brake on unwinding.

    Evidence Single-molecule manipulation and biochemical unwinding kinetics (preprint)

    Open questions at the time
    • Preprint, not peer-reviewed
    • Competition model not validated in a fully reconstituted replisome or in cells

Open questions

Synthesis pass · forward-looking unresolved questions
  • How SSBP1's moonlighting activities (nuclear chaperone co-activation, RNA-granule processing, ssDNA repair gating) are coordinated with its replisome role, and how its expression is wired into stress, immune, and oncogenic signaling, remains unresolved.
  • No mechanism partitions SSBP1 between mitochondrial and extramitochondrial pools
  • Signaling-context findings (DNA-PK/p53, MAVS/Smurf1, HMGB3, STAT3/FOXP1) are individual disease-context observations not unified mechanistically

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0098772 molecular function regulator activity 4 GO:0003677 DNA binding 3 GO:0003723 RNA binding 1
Localization
GO:0005739 mitochondrion 3 GO:0005634 nucleus 1
Pathway
R-HSA-69306 DNA Replication 2 R-HSA-8953854 Metabolism of RNA 1 R-HSA-8953897 Cellular responses to stimuli 1
Partners
AAGBRG1HSF1POLGTWNK
Complex memberships
mitochondrial RNA granule / GRSF1-mtRNA degradosomemtDNA replisome

Evidence

Reading pass · 18 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2015 Heat shock triggers nuclear translocation of mitochondrial SSBP1 in a manner dependent on the mitochondrial permeability transition pore ANT-VDAC1 complex and direct interaction with HSF1. In the nucleus, SSBP1 is recruited by HSF1 to promoters of genes encoding cytoplasmic/nuclear and mitochondrial chaperones, where the HSF1-SSBP1 complex enhances gene induction by facilitating recruitment of chromatin-remodelling factor BRG1, thereby supporting cell survival and mitochondrial membrane potential against proteotoxic stresses. Co-immunoprecipitation, chromatin immunoprecipitation (ChIP), live-cell imaging of nuclear translocation, loss-of-function experiments with specific phenotypic readouts (cell survival, mitochondrial membrane potential) Nature communications High 25762445
2011 Human mtSSB uses distinct structural surface elements (removed from the ssDNA-binding groove) to functionally interact with and stimulate DNA polymerase γ (pol γ) and the mtDNA helicase independently; variants defective in stimulating pol γ retained helicase stimulation capacity and vice versa. Overexpression of defective variants in Drosophila S2 cells caused mtDNA depletion, confirming these functional interactions are required for proper mtDNA replication in animal cells. In vitro DNA polymerase activity assay, in vitro DNA unwinding assay, site-directed mutagenesis of surface residues, mtDNA copy number measurement in cultured cells with overexpression of variants The Journal of biological chemistry High 21953457
2013 Alkyladenine DNA glycosylase (AAG) localizes to mitochondria and directly interacts with mtSSB (SSBP1). This interaction specifically inhibits AAG glycosylase activity on single-stranded DNA substrates but not double-stranded DNA substrates, and the interaction increases upon alkylating agent treatment. A putative surface motif on mtSSB may recruit UNG1 to DNA-bound mtSSB, potentially facilitating rapid processing of uracil once the dsDNA conformation is restored. Immunofluorescence localization, purification of mitochondrial extracts, direct binding assay (pulldown), in vitro glycosylase activity assay with ssDNA and dsDNA substrates DNA repair Medium 23290262
2011 mtSSB (SSBP1) impedes uracil excision and oxidative demethylation of 3meC in single-stranded DNA by UNG1 and ABH1 respectively, and partially inhibits NEIL1-mediated excision. mtSSB also effectively inhibited nicking of ssDNA by APE1 and ABH1 and partially inhibited the lyase activity of NEIL1, suggesting it prevents formation of DNA breaks in ssDNA during replication. In vitro base excision and DNA repair enzyme activity assays with purified mtSSB and ssDNA/dsDNA substrates DNA repair Medium 22153281
2019 mtSSB (SSBP1) is not restricted to nucleoids but also localizes to mitochondrial RNA granules. Depletion of mtSSB results in RNA processing defects, accumulation of mtRNA breakdown products, and increased levels of dsRNA and RNA:DNA hybrids, indicating that mtSSB participates in the GRSF1-mtRNA degradosome pathway for degradation of G-quadruplex-prone long non-coding mtRNAs. Immunofluorescence colocalization, siRNA-mediated depletion, RNA processing analysis, dsRNA detection, RNA:DNA hybrid detection Nucleic acids research Medium 30715486
2020 Missense mutations in SSBP1 (R38Q and R107Q) affect dimer/tetramer formation and impair mtDNA replication, leading to mtDNA depletion. Crystal structure of SSBP1 revealed that both mutated arginine residues affect dimer interactions and distort the DNA-binding region. Patient fibroblasts validated that R38Q destabilizes SSBP1 dimer/tetramer formation and reduces mtDNA replication efficiency. Reduced mtDNA replication was also reproduced in vitro. Crystal structure determination, size exclusion chromatography (oligomeric state), patient-derived fibroblast assays, in vitro mtDNA replication assay, zebrafish ssbp1 knockdown rescue experiments The Journal of clinical investigation High 31550237 31550240
2019 SSBP1 mutations R38Q, R107Q, and S141N affect arginine residues in the basic patch essential for single-strand DNA binding. Antisense-mediated knockdown of ssbp1 in zebrafish compromised differentiation of retinal ganglion cells, and a similar effect was achieved with mutated mRNAs, demonstrating dominant-negative effects of the disease variants. In silico structural analysis, zebrafish antisense knockdown, mRNA rescue experiments with wild-type and mutant mRNAs Annals of neurology Medium 31298765
2018 SSBP1 was identified as a putative binding protein for N6-methyldeoxyadenosine (6mA) on mitochondrial DNA heavy-strand using 6mACE-seq crosslinking-based pulldown, linking 6mA modification with regulation of mtDNA replication by SSBP1. 6mACE-seq (6mA-Crosslinking-Exonuclease-sequencing), genome-wide 6mA mapping, protein identification Nucleic acids research Low 30412255
2007 C. elegans par2.1/mtssb-1 (ortholog of SSBP1) is essential for mtDNA replication and germline cell proliferation; RNAi depletion over generations caused sterility with arrested germline cell proliferation, reduced mitochondrial number, comprehensive transcriptional alterations including hypoxia response, and reduced apoptosis frequency in germline cells. RNAi depletion across generations, mtDNA copy number measurement, cell cytology, transcriptome microarray analysis Experimental cell research Medium 17900564
2022 SSBP1 promotes ferroptosis in glomerular podocytes under high fructose conditions by interacting with DNA-dependent protein kinase (DNA-PK) and p53, activating DNA-PK to phosphorylate p53 at serine 15, promoting nuclear accumulation of p53 and subsequent inhibition of SLC7A11 expression. Co-immunoprecipitation (SSBP1-DNA-PK-p53 complex), siRNA knockdown, western blotting for p53 phosphorylation, nuclear fractionation, ferroptosis assays Redox biology Medium 35390676
2018 mtSSB (SSBP1) expression in colorectal cancer is induced by IL-6/STAT3 signaling via upregulation of the transcription factor FOXP1, which was identified as a new transcriptional regulator of mtSSB. Elevated mtSSB increased mitochondrial biogenesis and ROS production, which induced TERT expression and telomere elongation via the Akt/mTOR pathway. Reporter assays, ChIP for FOXP1 binding to mtSSB promoter, overexpression/knockdown, in vitro proliferation assays, in vivo xenograft, telomerase activity assays International journal of cancer Medium 30415472
2024 The disease-associated R107Q mutation in mtSSB (SSBP1) does not destabilize tetramers in vitro, but significantly reduces intramolecular ssDNA compaction ability and increases ssDNA dissociation rate compared to wild-type. Real-time competition experiments showed a marked advantage of wild-type mtSSB over R107Q mutant for ssDNA binding. Molecular modeling suggested R107Q creates an electronegative spot that disrupts an ssDNA-interacting electropositive patch, reducing potential mtSSB-ssDNA interaction sites. Single-molecule manipulation/visualization, in vitro ssDNA compaction assay, ssDNA dissociation kinetics measurement, real-time competition binding assay, molecular modeling Nucleic acids research High 38742632
2021 Molecular dynamics simulations of wild-type and 31 variant SSBP1 tetramers showed that all disease-associated variants form stable tetramers with stronger intermonomer interactions, reduced solvent accessible surface areas, and net loss of positive surface charge. Structural modeling identified potential DNA binding surfaces and hotspots, suggesting disease variants alter DNA binding/wrapping rather than abolishing binding altogether or destabilizing tetramers. Molecular dynamics simulations, structural alignment, phosphate binding simulations DNA repair Low 34464898
2019 A heterozygous start loss mutation in SSBP1, co-segregating with hearing loss in a multigenerational family carrying the m.1555A>G mtDNA variant, reduced steady-state SSBP1 protein levels and caused mtDNA depletion and multiple deletions in skeletal muscle, demonstrating that SSBP1 haploinsufficiency can compound an intra-mitochondrial translation defect in a tissue-specific manner. Exome sequencing, SSBP1 protein level quantification by western blot, mtDNA copy number and deletion analysis in skeletal muscle biopsy Brain : a journal of neurology Medium 29182774
2024 HMGB3 recruits and interacts with SSBP1 (demonstrated by co-immunoprecipitation combined with mass spectrometry), inducing SSBP1 nuclear translocation. This nuclear translocation reprograms mitochondrial metabolism, elevates cytoplasmic ROS, and activates the PI3K/Akt signaling pathway through PTEN downregulation, promoting tumor cell EMT and brain metastasis. Co-immunoprecipitation with mass spectrometry, western blotting, nuclear fractionation, gain-of-function and loss-of-function experiments, in vivo brain metastasis model Cancer communications (London, England) Medium 41194553
2025 SSBP1 promotes K48-linked ubiquitination of MAVS (mitochondrial antiviral signaling protein) by recruiting Smurf1 E3 ubiquitin ligase, thereby promoting proteasomal degradation of MAVS and suppressing antiviral innate immune responses during bovine ephemeral fever virus infection. Co-immunoprecipitation, ubiquitination assay (K48-linkage specific), proteasome inhibitor rescue, SSBP1 knockdown/overexpression with viral replication readout Veterinary microbiology Medium 40848354
2024 mtSSB (SSBP1) binding to DNA likely outcompetes the Zinc-binding domain (ZBD) of the Twinkle helicase for DNA interactions, alleviating ZBD-mediated downregulation of Twinkle's DNA unwinding kinetics. This places mtSSB as a positive regulator of Twinkle helicase activity at the mtDNA replication fork by relieving an auto-inhibitory constraint. Single-molecule manipulation and visualization, biochemical unwinding assays, real-time kinetics measurements bioRxivpreprint Medium
2023 STAT3 acts as a transcriptional repressor of SSBP1; chromatin immunoprecipitation (ChIP) and dual luciferase reporter assays demonstrated that STAT3 binds the SSBP1 promoter region and inhibits SSBP1 transcription. The compound sanguinarine inhibits JAK/STAT3 signaling, relieving STAT3 repression of SSBP1 and increasing SSBP1 expression, which disrupts mitochondrial function and induces apoptosis in osteosarcoma cells. Chromatin immunoprecipitation (ChIP), dual luciferase reporter assay, western blotting, flow cytometry (apoptosis, cell cycle, ROS) British journal of pharmacology Medium 37501645

Source papers

Stage 0 corpus · 50 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2002 Differentiation of prion protein glycoforms from naturally occurring sheep scrapie, sheep-passaged scrapie strains (CH1641 and SSBP1), bovine spongiform encephalopathy (BSE) cases and Romney and Cheviot breed sheep experimentally inoculated with BSE using two monoclonal antibodies. Acta neuropathologica 170 12172914
2015 Mitochondrial SSBP1 protects cells from proteotoxic stresses by potentiating stress-induced HSF1 transcriptional activity. Nature communications 99 25762445
1995 Chromosomal localization of mitochondrial transcription factor A (TCF6), single-stranded DNA-binding protein (SSBP), and endonuclease G (ENDOG), three human housekeeping genes involved in mitochondrial biogenesis. Genomics 84 7789991
2020 SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder. The Journal of clinical investigation 78 31550240
2018 Single-nucleotide-resolution sequencing of human N6-methyldeoxyadenosine reveals strand-asymmetric clusters associated with SSBP1 on the mitochondrial genome. Nucleic acids research 70 30412255
2015 RETRACTED: SSBP1 Suppresses TGFβ-Driven Epithelial-to-Mesenchymal Transition and Metastasis in Triple-Negative Breast Cancer by Regulating Mitochondrial Retrograde Signaling. Cancer research 67 26676758
2020 Dominant mutations in mtDNA maintenance gene SSBP1 cause optic atrophy and foveopathy. The Journal of clinical investigation 59 31550237
2021 The circular RNA circZFR phosphorylates Rb promoting cervical cancer progression by regulating the SSBP1/CDK2/cyclin E1 complex. Journal of experimental & clinical cancer research : CR 58 33516252
2022 SSBP1 drives high fructose-induced glomerular podocyte ferroptosis via activating DNA-PK/p53 pathway. Redox biology 56 35390676
2002 New Zealand sheep with scrapie-susceptible PrP genotypes succumb to experimental challenge with a sheep-passaged scrapie isolate (SSBP/1). The Journal of general virology 54 11961280
2019 SSBP1 mutations in dominant optic atrophy with variable retinal degeneration. Annals of neurology 53 31298765
2019 Mitochondrial RNA granules are critically dependent on mtDNA replication factors Twinkle and mtSSB. Nucleic acids research 52 30715486
2011 Reduced stimulation of recombinant DNA polymerase γ and mitochondrial DNA (mtDNA) helicase by variants of mitochondrial single-stranded DNA-binding protein (mtSSB) correlates with defects in mtDNA replication in animal cells. The Journal of biological chemistry 45 21953457
2015 Thermostable chitinase II from Thermomyces lanuginosus SSBP: Cloning, structure prediction and molecular dynamics simulations. Journal of theoretical biology 43 25861869
2019 Mitochondrial single-stranded DNA binding protein novel de novo SSBP1 mutation in a child with single large-scale mtDNA deletion (SLSMD) clinically manifesting as Pearson, Kearns-Sayre, and Leigh syndromes. PloS one 41 31479473
2022 Identification of SSBP1 as a ferroptosis-related biomarker of glioblastoma based on a novel mitochondria-related gene risk model and in vitro experiments. Journal of translational medicine 36 36180956
2013 Alkyladenine DNA glycosylase (AAG) localizes to mitochondria and interacts with mitochondrial single-stranded binding protein (mtSSB). DNA repair 31 23290262
1991 Primary structure of the two variants of Xenopus laevis mtSSB, a mitochondrial DNA binding protein. Archives of biochemistry and biophysics 29 1952953
2017 Downregulation of Mitochondrial Single Stranded DNA Binding Protein (SSBP1) Induces Mitochondrial Dysfunction and Increases the Radiosensitivity in Non-Small Cell Lung Cancer Cells. Journal of Cancer 26 28638454
1999 Purification and biochemical characteristics of beta-D-glucosidase from a thermophilic fungus, Thermomyces lanuginosus-SSBP. Biotechnology and applied biochemistry 26 10467123
2007 Caenorhabditis elegans par2.1/mtssb-1 is essential for mitochondrial DNA replication and its defect causes comprehensive transcriptional alterations including a hypoxia response. Experimental cell research 25 17900564
2014 Secretome analysis of the thermophilic xylanase hyper-producer Thermomyces lanuginosus SSBP cultivated on corn cobs. Journal of industrial microbiology & biotechnology 23 25223615
2018 Heterozygous SSBP1 start loss mutation co-segregates with hearing loss and the m.1555A>G mtDNA variant in a large multigenerational family. Brain : a journal of neurology 21 29182774
2018 Upregulation of mtSSB by interleukin-6 promotes cell growth through mitochondrial biogenesis-mediated telomerase activation in colorectal cancer. International journal of cancer 21 30415472
2023 Sanguinarine induces apoptosis in osteosarcoma by attenuating the binding of STAT3 to the single-stranded DNA-binding protein 1 (SSBP1) promoter region. British journal of pharmacology 19 37501645
1999 Purification and biochemical characteristics of beta-D-xylanase from a thermophilic fungus, Thermomyces lanuginosus-SSBP. Biotechnology and applied biochemistry 18 10467122
2012 Borrelia burgdorferi cp32 BpaB modulates expression of the prophage NucP nuclease and SsbP single-stranded DNA-binding protein. Journal of bacteriology 17 22730122
2019 SSBP1 Upregulation In Colorectal Cancer Regulates Mitochondrial Mass. Cancer management and research 16 31819642
2011 mtSSB may sequester UNG1 at mitochondrial ssDNA and delay uracil processing until the dsDNA conformation is restored. DNA repair 15 22153281
1992 Plasma sex steroid binding proteins (SSBP) in the male lizard, Podarcis s. sicula, during the reproductive cycle. General and comparative endocrinology 14 1398017
2021 SSBP1-Disease Update: Expanding the Genetic and Clinical Spectrum, Reporting Variable Penetrance and Confirming Recessive Inheritance. Investigative ophthalmology & visual science 13 34905022
2000 The production of hemicellulases by Thermomyces lanuginosus strain SSBP: influence of agitation and dissolved oxygen tension. Applied microbiology and biotechnology 11 11131398
2023 Structural basis of the interaction between BCL9-Pygo and LDB-SSBP complexes in assembling the Wnt enhanceosome. Nature communications 10 37349336
2021 Mechanisms of SSBP1 variants in mitochondrial disease: Molecular dynamics simulations reveal stable tetramers with altered DNA binding surfaces. DNA repair 10 34464898
2011 Transcriptional profiling of peripheral lymphoid tissue reveals genes and networks linked to SSBP/1 scrapie pathology in sheep. Veterinary microbiology 9 21684093
2021 De Novo Development of mtDNA Deletion Due to Decreased POLG and SSBP1 Expression in Humans. Genes 8 33671400
2024 Discovery of novel disease-causing mutation in SSBP1 and its correction using adenine base editor to improve mitochondrial function. Molecular therapy. Nucleic acids 6 39104869
2024 SSBP1 positively regulates RRM2, affecting epithelial mesenchymal transition and cell cycle arrest in human lung adenocarcinoma cells. Cellular signalling 6 39643024
2020 SSBP1 faux pas in mitonuclear tango causes optic neuropathy. The Journal of clinical investigation 5 31738184
2023 Maternal mosaicism in SSBP1 causing optic atrophy with retinal degeneration: implications for genetic counseling. Orphanet journal of rare diseases 4 37259171
2014 Transcriptome analysis of CNS immediately before and after the detection of PrP(Sc) in SSBP/1 sheep scrapie. Veterinary microbiology 3 25183238
2022 Case report: Monoclonal CGRP-antibody treatment in a migraine patient with a mutation in the mitochondrial single-strand binding protein (SSBP1). Frontiers in neurology 2 36203974
2025 SSBP1 promotes bovine ephemeral fever virus replication by antagonizing antiviral immune responses via degrading MAVS. Veterinary microbiology 1 40848354
2025 HMGB3 promotes brain metastasis of lung adenocarcinoma by recruiting SSBP1 for nuclear translocation to remodel mitochondrial metabolism. Cancer communications (London, England) 1 41194553
2025 Loss of mitochondrial single stranded DNA-binding protein (mtSSB) gene is associated with mitochondrial genome fragmentation in Psocodea (bark lice, book lice, and parasitic lice). BMC biology 1 41351112
2024 The mutation R107Q alters mtSSB ssDNA compaction ability and binding dynamics. Nucleic acids research 1 38742632
2022 The importance of genome sequencing: unraveling SSBP1 variant missed by exome sequencing. Ophthalmic genetics 1 35946466
2026 Mitochondrial transplantation restores mitochondrial content and function in SSBP1-related mitochondrial DNA depletion syndrome. BMB reports 0 41261791
2025 Inhibiting SSBP1 enhances ferroptosis and improves the effectiveness of sorafenib treatment for liver cancer. International journal of oncology 0 40747667
2023 Maternal Mosaicism in SSBP1 Causing Optic Atrophy with Retinal Degeneration: Implications for Genetic Counseling. Research square 0 36993412

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