Affinage

SUPV3L1

ATP-dependent RNA helicase SUPV3L1, mitochondrial · UniProt Q8IYB8

Length
786 aa
Mass
88.0 kDa
Annotated
2026-06-10
35 papers in source corpus 29 papers cited in narrative 28 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SUPV3L1 (SUV3) is a nuclear-encoded, ATP-dependent RNA/DNA helicase of the SF2 family that is the central determinant of mitochondrial post-transcriptional RNA metabolism, first defined in yeast where loss-of-function alters splicing and stability of intron-containing mitochondrial transcripts and the protein carries a mitochondrial targeting presequence with conserved helicase motifs (PMID:1379722, PMID:2158076, PMID:8529267). Crystal and cryo-EM structures define a four-domain architecture — two RecA-like domains, a C-terminal helical domain through which substrate RNA threads, and an external N-terminal domain — that assembles into an asymmetric homodimer via its C-terminal tail, an arrangement required for efficient, ATP-hydrolysis-dependent processive RNA binding and unwinding (PMID:22101826, PMID:35481630, PMID:41986356). As the catalytic core of the mitochondrial degradosome, the SUV3 dimer binds the PNPase trimer (through SUV3 residues ~510-514) to form a 330-kDa heteropentamer that degrades structured and double-stranded RNA with 3'→5' directionality, a reaction that requires intact helicase activity (PMID:19509288); the same complex transiently recruits mtPAP (via SUV3 residues ~100-104) to bidirectionally tune mt-mRNA poly(A) tail length in response to mitochondrial Pi/ATP ratios (PMID:24770417). Through these activities SUV3 controls processing of mitochondrial polycistronic transcripts, mt-mRNA and tRNA maturation, mtDNA copy number and replication, and overall mitochondrial bioenergetic function (PMID:18678873, PMID:21911497, PMID:26152302). SUV3 also degrades mitochondria-encoded circular RNAs together with ELAC2, and its loss causes accumulation of mitochondrial double-stranded RNAs that escape to the cytosol and activate PKR-mediated innate immune and type I interferon signaling, while mtDNA leakage drives PD-L1 upregulation (PMID:39973625, PMID:40268748, PMID:39267568). A distinct nuclear/nucleolar pool of SUV3 interacts with the BLM and WRN helicases and with RPA and FEN1 and suppresses homologous recombination, contributing to genome stability (PMID:17961633, PMID:21846330, PMID:28291845). In vivo, Supv3L1 is essential: conditional disruption causes premature aging and skin barrier failure, and heterozygous mice accumulate maternally transmitted mtDNA mutations with reduced copy number and develop tumors (PMID:19145458, PMID:22562243). A homozygous truncating SUPV3L1 mutation causes a human mitochondrial RNA-processing disorder with reduced mature ND6 mRNA and dsRNA accumulation, rescued by cDNA complementation (PMID:35023579).

Mechanistic history

Synthesis pass · year-by-year structured walk · 20 steps
  1. 1992 High

    Established the molecular identity of SUV3 as a mitochondrial ATP-dependent RNA helicase and linked a point mutation in a conserved helicase motif to mitochondrial RNA defects, defining the gene's biochemical class.

    Evidence Gene cloning, sequencing, and mutant-allele analysis of the SUV3-1 gain-of-function mutation in yeast

    PMID:1379722 PMID:2158076

    Open questions at the time
    • Helicase catalytic activity not demonstrated biochemically on purified protein
    • Direct RNA substrates not defined
  2. 1995 High

    Showed SUV3 is required for stability and processing of intron-containing mitochondrial transcripts in an intron-number-dependent manner, framing it as a post-transcriptional RNA stability factor rather than a transcriptional regulator.

    Evidence Northern analysis in yeast strains with defined intron combinations plus SUV3 disruption

    PMID:7736607 PMID:8529267

    Open questions at the time
    • Mechanism distinguishing direct turnover from indirect effects not resolved
    • rRNA processing role from single intron background
  3. 1999 Medium

    Identified human SUPV3L1 as the conserved orthologue with a mitochondrial leader sequence, extending the yeast function to a candidate human mitochondrial helicase.

    Evidence cDNA cloning, sequencing, Northern and EST analysis

    PMID:10453991

    Open questions at the time
    • No functional assay of the human protein
    • Subcellular localization inferred from sequence only
  4. 2007 Medium

    Revealed an unexpected nuclear pool of human SUV3 and its interaction with RecQ helicases BLM and WRN, implicating it in genome stability and HR suppression beyond mitochondria.

    Evidence siRNA knockdown with apoptosis/cell-cycle assays, ELISA binding, and sister chromatid exchange assays in HeLa cells

    PMID:17352692 PMID:17961633

    Open questions at the time
    • Co-IP/ELISA without structural mapping of interaction interfaces
    • Whether nuclear effects are direct or secondary to mitochondrial dysfunction unresolved
  5. 2008 High

    Demonstrated that loss of human SUV3 causes shortened polyadenylated mt-RNAs and broad bioenergetic collapse, establishing its essential role in mammalian mitochondrial RNA metabolism and cell viability.

    Evidence siRNA knockdown with mt-RNA, protein synthesis, ROS, membrane potential, mtDNA copy number, and morphology assays

    PMID:18678873

    Open questions at the time
    • Direct vs. indirect cause of poly(A) shortening not separated
    • Enzymatic mechanism not addressed
  6. 2009 High

    Reconstituted the mitochondrial degradosome, showing the SUV3 dimer and PNPase trimer form a 330-kDa heteropentamer that degrades dsRNA 3'→5' in an ATP- and helicase-activity-dependent manner.

    Evidence Purified protein reconstitution, gel filtration sizing, in vitro RNA degradation, and site-directed mutagenesis (residues 510-514)

    PMID:19509288

    Open questions at the time
    • In vivo substrate repertoire not defined
    • Regulation of complex assembly in cells unknown
  7. 2009 High

    Showed in vivo that Supv3L1 is organismally essential, with conditional loss causing premature aging and skin barrier failure.

    Evidence Conditional Cre/lox disruption with histology and phenotyping in mice

    PMID:19145458

    Open questions at the time
    • Molecular link between mitochondrial RNA defects and skin phenotype not delineated
    • Tissue-specific substrate dependencies unknown
  8. 2010 High

    Resolved the mechanism of SUV3's splicing role as indirect, via degradosome-mediated turnover of excised intron RNPs that recycle a limiting splicing cofactor.

    Evidence Genetic epistasis with suv3-1, Mrs1p overexpression/depletion, and intron RNP analysis in yeast

    PMID:20064926

    Open questions at the time
    • Generality of cofactor-sequestration model to other introns/organisms untested
  9. 2011 High

    Separated SUV3's mtDNA maintenance function from intron turnover and tied it to active replication origins, requiring ATPase activity and the CC region but not RNA binding.

    Evidence Inducible knockdown with structure-function mutagenesis (K245A, V272L, ΔCC) and ChIP at mtDNA origins in yeast

    PMID:21911497

    Open questions at the time
    • Direct DNA substrate at origins not biochemically defined
    • How helicase activity supports replication mechanistically unresolved
  10. 2011 Medium

    Connected nuclear SUV3 to replication/repair machinery by demonstrating physical and functional interplay with RPA and FEN1.

    Evidence Co-IP of hSuv3 complexes, in vitro helicase assays with RPA vs mtSSB, and FEN1 stimulation assays

    PMID:21846330

    Open questions at the time
    • Single Co-IP without reciprocal in-cell validation of functional consequence
    • No demonstration that SUV3 acts at nuclear replication forks in vivo
  11. 2011 High

    Provided the first atomic-resolution architecture of human Suv3, defining a four-domain SF2 fold with an RNA-threading C-terminal domain and assigning it to a distinct helicase subfamily.

    Evidence X-ray crystallography with AMPPNP and RNA ligands plus functional validation

    PMID:22101826

    Open questions at the time
    • Oligomeric/dimeric state and its functional role not captured in monomeric structures
  12. 2012 High

    Established a causal in vivo link between SUV3 dosage and mtDNA integrity, with haploinsufficiency producing maternally transmitted mtDNA mutations, copy-number loss, tumorigenesis, and shortened lifespan.

    Evidence Heterozygous mouse genetics with mtDNA mutation/copy-number analysis, tumor histology, and maternal transmission

    PMID:22562243

    Open questions at the time
    • Mechanistic chain from mtDNA mutation to specific tumor types not defined
  13. 2014 High

    Defined a regulatory function for the degradosome in adjusting mt-mRNA poly(A) tail length, with SUV3 simultaneously bridging mtPAP and PNPase to sense the mitochondrial Pi/ATP ratio.

    Evidence Reconstitution of the SUV3·PNPase·mtPAP complex, binding-site mutagenesis (residues 100-104), polyadenylation/degradation assays, and cellular Pi manipulation

    PMID:24770417

    Open questions at the time
    • Physiological signals controlling complex switching in vivo not fully mapped
    • Which mt-mRNAs are preferentially regulated unresolved
  14. 2014 Medium

    Characterized cofactor dependence of human Suv3, showing helicase activity requires specific divalent cations and that only ATP supports activity at high nucleotide concentrations, implying sensitivity to local metabolite availability.

    Evidence In vitro helicase and ATPase assays with varied divalent cations and nucleotides

    PMID:25446650

    Open questions at the time
    • In vitro biochemistry; physiological relevance of cofactor sensitivity untested
    • Single method type
  15. 2015 High

    Extended SUV3 function to metazoan mitochondrial polycistronic transcript processing, showing tRNA maturation and translation defects that occur independently of PNPase.

    Evidence Drosophila loss-of-function genetics with Northern blotting, translation, and respiratory complex assays

    PMID:26152302

    Open questions at the time
    • PNPase-independent processing mechanism not biochemically defined
  16. 2017 Medium

    Localized human SUV3 to nucleoli and attributed the growth defect of SUV3 depletion to nuclear rather than mitochondrial function, while excluding it from DNA-repair foci.

    Evidence Fluorescence microscopy with nuclear-targeted constructs and siRNA growth assays in HeLa cells

    PMID:28291845

    Open questions at the time
    • Nucleolar substrate/function of SUV3 unidentified
    • Reconciliation with reported HR/repair-factor interactions unresolved
  17. 2022 High

    Defined dimerization via the C-terminal tail as mechanistically required for efficient RNA unwinding and PNPase binding, and described the dumbbell-shaped degradosome architecture.

    Evidence CTT mutagenesis, in vitro RNA binding/unwinding assays, crystal structure of Suv3ΔC, and SAXS of dimeric and PNPase-bound complexes

    PMID:35481630

    Open questions at the time
    • High-resolution structure of the full dimeric degradosome not yet resolved at this stage
  18. 2022 Medium

    Provided human disease evidence that biallelic SUPV3L1 loss causes a mitochondrial RNA-processing disorder, with cDNA complementation confirming pathogenicity.

    Evidence Patient fibroblast RNA analysis, dsRNA immunostaining, and lentiviral cDNA complementation rescue

    PMID:35023579

    Open questions at the time
    • Only two siblings; phenotypic spectrum and genotype-phenotype range undefined
    • Partial rescue leaves residual mechanism uncertain
  19. 2024 Medium

    Linked SUV3 loss to innate immune dysregulation, showing accumulated mitochondrial dsRNA escapes to the cytosol and activates PKR-driven type I IFN signaling, and that mtDNA leakage drives PD-L1 upregulation.

    Evidence siRNA knockdown, fCLIP-qPCR, TEM, cytokine and functional assays with PKR co-knockdown rescue; mtDNA fractionation with TREX1 overexpression rescue

    PMID:39267568 PMID:39973625 PMID:40268748

    Open questions at the time
    • Single lab per pathway arm
    • Direct mechanism of dsRNA/mtDNA cytosolic escape not fully defined
  20. 2026 High

    Defined the structural basis of processive unwinding by capturing asymmetric dimeric Suv3 in four functional states, showing only one protomer initially engages nucleotide/RNA in an ATP-hydrolysis-dependent cycle.

    Evidence Cryo-EM in apo, ADP, ssRNA, and ssRNA+AMP-PNP states with biochemical RNA-binding/unwinding validation

    PMID:41986356

    Open questions at the time
    • Structure of the full degradosome engaging substrate not resolved
    • Coupling of unwinding to PNPase degradation step not visualized

Open questions

Synthesis pass · forward-looking unresolved questions
  • How the same protein partitions and coordinates its distinct mitochondrial RNA-degradation/processing roles, its nucleolar/HR-related nuclear functions, and its mtDNA-replication role remains unresolved, as does the in vivo substrate map across compartments.
  • No unified model linking nuclear and mitochondrial pools
  • Nucleolar substrate undefined
  • Full in vivo mt-RNA substrate repertoire incomplete

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140098 catalytic activity, acting on RNA 5 GO:0003723 RNA binding 4 GO:0140657 ATP-dependent activity 4 GO:0003677 DNA binding 2 GO:0016787 hydrolase activity 2
Localization
GO:0005739 mitochondrion 4 GO:0005634 nucleus 2 GO:0005730 nucleolus 1
Pathway
R-HSA-8953854 Metabolism of RNA 4 R-HSA-168256 Immune System 1 R-HSA-69306 DNA Replication 1
Partners
BLMELAC2FEN1GRSF1MTPAPPNPT1RPAWRN
Complex memberships
SUPV3L1-ELAC2 complexSUV3-PNPase-mtPAP complexmitochondrial degradosome (SUV3-PNPase)

Evidence

Reading pass · 28 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1992 The yeast SUV3 gene encodes an 84-kDa protein with a mitochondrial targeting presequence and conserved sequence motifs of ATP-dependent RNA helicases; the SUV3-1 gain-of-function mutation is a Val→Leu substitution in a conserved helicase motif block, causing accumulation of excised group I introns in mitochondria. Gene cloning by colony Northern hybridization, sequence analysis, and mutant allele identification Proceedings of the National Academy of Sciences of the United States of America High 1379722
1990 The SUV3-1 mutation causes over-accumulation of excised group I intron RNAs, lowers levels of cob and cox1 mRNAs, and blocks dodecamer cleavage in yeast mitochondria, establishing SUV3 as a key regulator of mitochondrial post-transcriptional processes including RNA stability and splicing. Northern hybridization survey of mitochondrial RNAs in SUV3-1 and suv3 deletion strains Nucleic acids research High 2158076
1995 SUV3 is required for stability of intron-containing mitochondrial transcripts in a cumulative manner depending on intron number; loss of SUV3 decreases mRNA levels without accumulating high-molecular-weight precursors or affecting transcription. Northern hybridization of mitochondrial RNAs in strains with defined combinations of cytb and cox1 introns combined with SUV3 gene disruption Current genetics High 8529267
1995 The suv3 protein is required for proper processing of r1 intron-containing 21S rRNA transcripts; in its absence, the excised r1 intron pattern is altered and mature 21S rRNA is ~50-fold reduced. Northern analysis of mitochondrial RNA in suv3 disruption strain carrying r1-only intron genome Current genetics Medium 7736607
1998 The PET127 gene (involved in 5'-end processing of mitochondrial mRNAs) can suppress SUV3 or DSS1 gene disruptions, suggesting functional coupling between 5' and 3' ends of mitochondrial mRNAs and placing SUV3/DSS1 degradosome activity in a pathway coordinated with 5'-end processing. Genetic suppression by PET127 on low- and high-copy vectors in SUV3/DSS1 disruption strains Acta biochimica Polonica Medium 10397341
1999 Human SUPV3L1 (hSUV3) encodes a protein with a mitochondrial leader sequence that is the human homologue of yeast Suv3 helicase, conserved across eukaryotes and Rhodobacter sphaeroides. cDNA cloning, sequencing, Northern blot, and EST database analysis Acta biochimica Polonica Medium 10453991
2002 Overexpression of the yeast mitochondrial helicase Mss116 can partially suppress the loss of SUV3, restoring respiratory competence and normalizing COB and ATP6/8 mRNA levels, indicating functional overlap between these two distinct RNA helicase classes in mitochondrial RNA metabolism. Multicopy plasmid suppression, respiratory growth assays, and Northern blotting in SUV3-deleted strains Yeast (Chichester, England) Medium 12402239
2007 Human SUV3 localizes not only to mitochondria but also to the nucleus; siRNA-mediated knockdown in HeLa cells causes cell cycle perturbations, p53 induction, and both AIF-dependent and caspase-dependent apoptosis. siRNA knockdown, cell cycle analysis, apoptosis assays (AIF and caspase pathway), subcellular fractionation/localization Biology of the cell Medium 17352692
2007 Human SUV3 protein physically interacts with BLM helicase (Kd ~0.5 nM) and WRN helicase (Kd ~5 nM) as measured by ELISA binding assay; SUV3 knockdown in HeLa cells elevates sister chromatid exchange frequency, indicating a role in homologous recombination suppression. ELISA binding assay for protein-protein interaction, sister chromatid exchange assay after siRNA knockdown Mechanisms of ageing and development Medium 17961633
2008 Knockdown of hSUV3 in mammalian cells causes accumulation of shortened polyadenylated mitochondrial RNA species, impaired mitochondrial protein synthesis, increased reactive oxygen species, decreased membrane potential and ATP production, reduced mtDNA copy number, and shift of mitochondrial morphology from tubular to granular, leading to senescence or cell death. siRNA knockdown, mitochondrial RNA analysis, protein synthesis assay, ROS measurement, membrane potential assay, mtDNA copy number quantification, fluorescence microscopy of mitochondrial morphology The Journal of biological chemistry High 18678873
2009 Purified human SUV3 dimer and PNPase trimer form a 330-kDa heteropentamer (2 SUV3 + 3 PNPase) that degrades double-stranded RNA with 3'-to-5' directionality and preference for substrates with 3' overhang, in an ATP-dependent manner; deletion of SUV3 residues 510-514 abolishes stable complex formation and dsRNA degradation but not helicase activity; ATPase-dead or RNA-binding-dead SUV3 mutants can still bind PNPase but the resulting complexes fail to degrade dsRNA, establishing that intact helicase activity is essential for degradosome function. Purified protein reconstitution, gel filtration molecular sizing, in vitro RNA degradation assays, site-directed mutagenesis, biochemical interaction assays The Journal of biological chemistry High 19509288
2009 Conditional disruption of Supv3L1 in the mouse (using Mx1-Cre or tamoxifen-inducible Esr1/Cre) causes postnatal growth delay, loss of adipose tissue and muscle mass, severe skin abnormalities (ichthyosis, epidermal thickening, dermal atrophy), premature aging, and death; keratinocyte-specific ablation confirms atrophic skin changes, establishing Supv3L1 as essential for skin barrier maintenance. Conditional gene disruption (Cre/lox), histology, and phenotypic analysis in mice Mammalian genome : official journal of the International Mammalian Genome Society High 19145458
2010 Yeast Suv3p acts indirectly through the degradosome (with Dss1p) to promote splicing of the aI5beta group I intron by degrading excised aI5beta ribonucleoprotein (RNP), thereby recycling the limiting splicing cofactor Mrs1p; sequestration of Mrs1p in stable excised intron RNPs accounts for reduced splicing in suv3 mutants. Genetic epistasis using suv3-1 allele (retains stable mtDNA), overexpression/depletion of Mrs1p, intron RNP analysis, and RNA metabolic assays The Journal of biological chemistry High 20064926
2011 SUV3 ATPase-dead (K245A) and CC-region deletion (ΔCC, residues ~511-518) mutants lose both degradosome activity and mtDNA maintenance; V272L (RNA-binding mutant) loses degradosome activity but retains mtDNA maintenance under intronless background; wild-type SUV3 and V272L, but not K245A or ΔCC, associate with active mtDNA replication origins and facilitate mtDNA replication, establishing a direct role for SUV3 in mtDNA maintenance independent of intron turnover that requires intact ATPase activity and the CC region. Inducible knockdown with structure-function mutagenesis in yeast, intronless mtDNA genetic backgrounds, chromatin immunoprecipitation at mtDNA replication origins The Journal of biological chemistry High 21911497
2011 Human hSuv3 physically interacts with RPA (replication protein A) and FEN1 (flap endonuclease 1) in the nucleus; low amounts of RPA inhibit hSuv3 helicase activity on forked substrates in vitro (while mitochondrial SSB does not, indicating specificity); hSuv3 stimulates FEN1 flap endonuclease activity in vitro independently of flap length. Co-immunoprecipitation of hSuv3 complexes, in vitro helicase activity assays with RPA or mtSSB, in vitro FEN1 endonuclease stimulation assay The Biochemical journal Medium 21846330
2011 X-ray crystal structures of human Suv3 in complex with the non-hydrolysable ATP analog AMPPNP (2.08 Å) and with a 5-nucleotide RNA strand (2.9 Å) reveal a four-domain architecture: two RecA-like domains (typical of SF2 helicases) forming a ring with a C-terminal all-helical domain through which the nucleotide strand threads, and an N-terminal helical domain positioned externally; unique structural features suggest Suv3 constitutes a separate helicase subfamily. X-ray crystallography with functional validation Acta crystallographica. Section D, Biological crystallography High 22101826
2012 Heterozygous mSuv3+/− mice accumulate increased mitochondrial DNA mutations and have decreased mtDNA copy numbers, leading to tumor development at multiple sites and shortened lifespan; these phenotypes are transmitted maternally, demonstrating a causative mitochondrial etiology. Mouse genetics (heterozygous knockout), mtDNA mutation analysis, mtDNA copy number quantification, tumor histology, maternal transmission analysis Oncogene High 22562243
2014 SUV3·PNPase complex interacts with mitochondrial polyadenylation polymerase (mtPAP) under low inorganic phosphate (Pi) conditions; SUV3 bridges mtPAP (via SUV3 N-terminal residues ~100-104) and PNPase (via SUV3 C-terminal residues ~510-514) simultaneously; SUV3 enhances mtPAP polyadenylation activity through its ssRNA binding domain; the reconstituted SUV3·PNPase·mtPAP complex (SUV3 dimer + mtPAP dimer + PNPase trimer) can lengthen or shorten mt-mRNA poly(A) tails depending on Pi/ATP ratios; poly(A) tail lengths in cells are modulated by altering mitochondrial matrix Pi via selective inhibition of respiratory chain or ATP synthase. Purified protein reconstitution, in vitro binding assays with defined SUV3 mutants, molecular sizing, in vitro polyadenylation and RNA degradation assays, cellular Pi manipulation with respiratory chain inhibitors The Journal of biological chemistry High 24770417
2014 Human hSuv3 helicase and ATPase activities are strictly dependent on specific divalent cations; multiple NTPs and dNTPs can support helicase activity at low concentrations (cofactor-dependent), but only ATP supports helicase activity at higher nucleotide concentrations, suggesting that hSuv3 DNA unwinding capacity is sensitive to local inorganic cofactor availability. In vitro biochemical helicase and ATPase assays with varied divalent cations and nucleotide cofactors Biochimie Medium 25446650
2015 Loss of dmsuv3 (Drosophila SUV3) causes accumulation of mitochondrial mRNAs without increasing rRNA levels, severe decrease in mitochondrial tRNAs with accumulation of unprocessed polycistronic precursor transcripts, reduced mitochondrial translation, respiratory chain complex deficiency, and pupal lethality; these processing defects occur independently of PNPase, establishing SUV3 as predominantly required for processing of mitochondrial polycistronic transcripts in metazoans. Drosophila loss-of-function genetics, Northern blotting, mitochondrial translation assay, respiratory chain complex activity measurement Nucleic acids research High 26152302
2017 Human SUV3 helicase localizes to HeLa cell nucleoli (in addition to mitochondria); nuclear-targeted SUV3 constructs establish that the cell growth rate impairment upon SUV3 depletion is due to nuclear (not mitochondrial) SUV3 function; SUV3 is not detectable in DNA-repair foci. Fluorescence microscopy with nuclear-targeted SUV3 constructs, siRNA knockdown with cell growth assays, co-localization with nucleolar markers Acta biochimica Polonica Medium 28291845
2018 The mitochondrial degradosome (SUV3·PNPase) together with the G-quadruplex-melting protein GRSF1 restricts mitochondrial antisense RNAs, including those forming G-quadruplex structures. Molecular and biochemical characterization of SUV3-PNPase-GRSF1 complex activity on antisense mt-RNAs Molecular & cellular oncology Low 30525095
2022 Dimeric assembly of human Suv3 via its C-terminal tail (CTT) is required for efficient RNA unwinding: CTT-truncated monomeric Suv3 (Suv3ΔC) has ~6-7-fold lower RNA binding and unwinding activities, cannot bind RNA independently of ATP/ADP, and fails to interact with PNPase; crystal structure of apo-Suv3ΔC and SAXS structures of dimeric Suv3 and PNPase-Suv3 complex show dimeric Suv3 caps on top of PNPase via S1 domain interactions forming a dumbbell-shaped degradosome. Mutagenesis of CTT domain, in vitro RNA binding and unwinding assays, crystal structure of truncation mutant, SAXS of native complexes, PNPase binding assay Protein science : a publication of the Protein Society High 35481630
2022 A homozygous truncating SUPV3L1 mutation in two siblings causes mitochondrial RNA processing defects including reduction of mature ND6 mRNA and accumulation of double-stranded RNA in patient fibroblasts; lentiviral complementation with full-length SUPV3L1 cDNA partly restores these RNA phenotypes, confirming pathogenicity. Patient fibroblast analysis, RT-PCR/Northern blot for mt-RNA processing, dsRNA immunostaining, lentiviral cDNA complementation Journal of inherited metabolic disease Medium 35023579
2025 SUPV3L1 and endoribonuclease ELAC2 form a conserved complex that rapidly degrades mitochondria-encoded circular RNAs (mecciRNAs); SUV3 knockdown leads to accumulation of mitochondrial dsRNAs that escape to the cytosol and activate PKR, triggering type I IFN signaling and proinflammatory cytokine production; this PKR activation accounts for impaired innate immune functions (migration, phagocytosis, ATP synthesis) in SUV3-deficient monocytes, reversible by PKR co-knockdown. Protein complex identification, siRNA knockdown, fCLIP-qPCR for dsRNA-PKR interaction, TEM for subcellular localization of dsRNAs, cytokine assays, functional assays (migration, phagocytosis, ATP synthesis), rescue by PKR co-knockdown Circulation / Rheumatology (Oxford, England) Medium 39973625 40268748
2024 SUV3 knockdown in hepatocellular carcinoma cells reduces mtDNA copy number, causes mtDNA leakage into the cytoplasm, and elevates PD-L1 expression; TREX1 overexpression in SUV3-knockdown cells reduces cytoplasmic mtDNA and suppresses the PD-L1 induction, establishing that cytoplasmic mtDNA accumulation mediates the PD-L1 upregulation. siRNA knockdown, subcellular fractionation for mtDNA quantification, qRT-PCR for PD-L1, TREX1 overexpression rescue experiment Zhonghua gan zang bing za zhi Medium 39267568
2026 Cryo-EM structures of human Suv3 in four functional states (apo, ADP-bound, ssRNA-bound, ssRNA+AMP-PNP-bound) at near-atomic resolution reveal an asymmetric dimeric organization in which only one protomer engages ADP, ssRNA, or both ssRNA and AMP-PNP in initial binding; biochemical analyses confirm that dimerization enhances RNA-binding and unwinding efficiency in an ATP-hydrolysis-dependent manner, establishing the mechanistic basis for processive RNA unwinding. Cryo-EM structure determination in four functional states, biochemical RNA-binding and unwinding assays with wild-type and mutant Suv3 Nature communications High 41986356
2024 Depletion of SUV3 helicase triggers formation of distinct mitochondrial RNA granules (termed inhibition granules) that differ from canonical mitochondrial RNA granules; these granules stabilize certain mt-mRNAs and appear to serve a protective function during transcription inhibition. Single-molecule RNA-FISH after SUV3 depletion, comparison with canonical MRG markers bioRxiv (preprint)preprint Low bio_10.1101_2024.09.25.614902

Source papers

Stage 0 corpus · 35 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1992 The yeast nuclear gene suv3 affecting mitochondrial post-transcriptional processes encodes a putative ATP-dependent RNA helicase. Proceedings of the National Academy of Sciences of the United States of America 91 1379722
2009 Human mitochondrial SUV3 and polynucleotide phosphorylase form a 330-kDa heteropentamer to cooperatively degrade double-stranded RNA with a 3'-to-5' directionality. The Journal of biological chemistry 88 19509288
1990 The nuclear SUV3-1 mutation affects a variety of post-transcriptional processes in yeast mitochondria. Nucleic acids research 59 2158076
2008 Role of SUV3 helicase in maintaining mitochondrial homeostasis in human cells. The Journal of biological chemistry 48 18678873
1999 A human putative Suv3-like RNA helicase is conserved between Rhodobacter and all eukaryotes. Acta biochimica Polonica 41 10453991
1995 The S. cerevisiae nuclear gene SUV3 encoding a putative RNA helicase is necessary for the stability of mitochondrial transcripts containing multiple introns. Current genetics 41 8529267
2012 Mitochondrial genome instability resulting from SUV3 haploinsufficiency leads to tumorigenesis and shortened lifespan. Oncogene 32 22562243
2007 Down-regulation of human RNA/DNA helicase SUV3 induces apoptosis by a caspase- and AIF-dependent pathway. Biology of the cell 31 17352692
2014 Helicase SUV3, polynucleotide phosphorylase, and mitochondrial polyadenylation polymerase form a transient complex to modulate mitochondrial mRNA polyadenylated tail lengths in response to energetic changes. The Journal of biological chemistry 29 24770417
2007 Interaction of human SUV3 RNA/DNA helicase with BLM helicase; loss of the SUV3 gene results in mouse embryonic lethality. Mechanisms of ageing and development 27 17961633
1995 The suv3 nuclear gene product is required for the in vivo processing of the yeast mitochondrial 21s rRNA transcripts containing the r1 intron. Current genetics 26 7736607
2015 SUV3 helicase is required for correct processing of mitochondrial transcripts. Nucleic acids research 22 26152302
2010 Splicing of yeast aI5beta group I intron requires SUV3 to recycle MRS1 via mitochondrial degradosome-promoted decay of excised intron ribonucleoprotein (RNP). The Journal of biological chemistry 20 20064926
1998 Yeast nuclear PET127 gene can suppress deletions of the SUV3 or DSS1 genes: an indication of a functional interaction between 3' and 5' ends of mitochondrial mRNAs. Acta biochimica Polonica 20 10397341
2025 Fast Degradation of MecciRNAs by SUPV3L1/ELAC2 Provides a Novel Opportunity to Tackle Heart Failure With Exogenous MecciRNA. Circulation 19 39973625
2018 Controlling the mitochondrial antisense - role of the SUV3-PNPase complex and its co-factor GRSF1 in mitochondrial RNA surveillance. Molecular & cellular oncology 19 30525095
2011 Uncoupling the roles of the SUV3 helicase in maintenance of mitochondrial genome stability and RNA degradation. The Journal of biological chemistry 19 21911497
2002 Overexpressed yeast mitochondrial putative RNA helicase Mss116 partially restores proper mtRNA metabolism in strains lacking the Suv3 mtRNA helicase. Yeast (Chichester, England) 19 12402239
2011 The human Suv3 helicase interacts with replication protein A and flap endonuclease 1 in the nucleus. The Biochemical journal 18 21846330
2009 Disruption of Supv3L1 damages the skin and causes sarcopenia, loss of fat, and death. Mammalian genome : official journal of the International Mammalian Genome Society 17 19145458
2011 Human Suv3 protein reveals unique features among SF2 helicases. Acta crystallographica. Section D, Biological crystallography 13 22101826
2022 Mitochondrial RNA processing defect caused by a SUPV3L1 mutation in two siblings with a novel neurodegenerative syndrome. Journal of inherited metabolic disease 12 35023579
2022 Dimeric assembly of human Suv3 helicase promotes its RNA unwinding function in mitochondrial RNA degradosome for RNA decay. Protein science : a publication of the Protein Society 11 35481630
2014 Rice SUV3 is a bidirectional helicase that binds both DNA and RNA. BMC plant biology 8 25311683
2004 The SUV3 gene from Saccharomyces douglasii is a functional equivalent of its Saccharomyces cerevisiae orthologue and is essential for respiratory growth. FEMS yeast research 7 14734028
2014 Salt tolerant SUV3 overexpressing transgenic rice plants conserve physicochemical properties and microbial communities of rhizosphere. Chemosphere 6 25303666
2016 Overexpression of PDH45 or SUV3 helicases in rice leads to delayed leaf senescence-associated events. Protoplasma 4 27586643
2009 Widespread expression of the Supv3L1 mitochondrial RNA helicase in the mouse. Transgenic research 3 19937380
2023 SUV3 Helicase and Mitochondrial Homeostasis. International journal of molecular sciences 2 37298184
2017 Human SUV3 helicase regulates growth rate of the HeLa cells and can localize in the nucleoli. Acta biochimica Polonica 2 28291845
2025 Diminished SUV3 expression and its functional implications in the IFN-enriched monocyte subset of childhood Sjögren's disease. Rheumatology (Oxford, England) 1 40268748
2024 A New Case of Mitochondrial RNA Helicase SUPV3L1-Associated Neurodegenerative Disease: Ataxia, Spasticity, Optic Atrophy, and Skin Hypopigmentation (ASOASH). Genes 1 39596606
2014 Regulation of the human Suv3 helicase on DNA by inorganic cofactors. Biochimie 1 25446650
2026 Asymmetric dimeric assembly of Suv3 helicase facilitates processive RNA unwinding. Nature communications 0 41986356
2024 [SUV3 knockdown inhibits proliferation, migration, and invasion of hepatocellular carcinoma cells and induces PD-L1 expression]. Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology 0 39267568

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