Affinage

HBS1L

HBS1-like protein · UniProt Q9Y450

Length
684 aa
Mass
75.5 kDa
Annotated
2026-06-10
71 papers in source corpus 17 papers cited in narrative 17 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

HBS1L is a translational GTPase that, together with its partner PELOTA (Dom34), recognizes and rescues stalled ribosomes to initiate mRNA surveillance pathways (PMID:20947765, PMID:23667253). The complex binds the ribosomal A-site in a manner that structurally mimics elongation factor-tRNA and eRF1-eRF3 termination complexes, and its nucleotide (GTP/GDP) binding drives ribosomal subunit dissociation and peptidyl-tRNA drop-off during no-go decay and 18S non-functional rRNA decay (PMID:20947765, PMID:21102444); the Dom34-Hbs1 interaction is required for no-go decay but dispensable for 18S NRD, genetically uncoupling these activities (PMID:21102444). HBS1L-mediated rescue acts downstream of ribosomal protein ubiquitination by ZNF-598 and feeds stalled transcripts into decay by the nuclease NONU-1 (PMID:36626369), and the shared N-terminal UBAh domain directly binds ubiquitin via its hydrophobic patch, providing the molecular basis for recruitment to ubiquitinated stalled ribosomes (PMID:42234679). Beyond no-go decay, HBS1L drives non-stop decay through a complex with the exosome and SKI machinery, with a short splice isoform (HBS1LV3/SKI7) bridging the cytoplasmic exosome (via its C-terminal RxxxFxxxL motif contacting RRP43) while the canonical PELOTA-binding isoform does not bind the exosome and antagonizes SKI/exosome supercomplex formation (PMID:23667253, PMID:28204585). The complex also splits inactive non-translating 80S ribosomes together with ABCE1 to enable translation restart after stress (PMID:24424461). Loss of HBS1L destabilizes PELOTA post-translationally via proteasomal degradation, causes 80S monosome accumulation, and activates mTOR and the unfolded protein response (PMID:30707697, PMID:41101730). These functions are essential in vivo: biallelic HBS1L loss causes developmental anomalies and retinal dystrophy with photoreceptor apoptosis in humans and mice (PMID:30707697, PMID:38966981), HBS1L is synthetic lethal with FOCAD loss in cancer and is the target of a cereblon-recruiting molecular glue degrader (TNG961) that triggers translational arrest and UPR in FOCAD-negative cells (PMID:41101730, PMID:42001523), and HBS1L knockdown in erythroid progenitors upregulates fetal hemoglobin (PMID:36888630).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2010 High

    Establishing the core biochemical activity: whether the Dom34:Hbs1 complex actively dissociates stalled ribosomes rather than merely binding them, and how this relates to canonical termination.

    Evidence Reconstituted yeast translation system with in vitro ribosome dissociation and peptidyl-tRNA drop-off assays; crystal structure of Hbs1 ± GDP with low-resolution Dom34-Hbs1 model and mutagenesis

    PMID:20947765 PMID:21102444

    Open questions at the time
    • Done in yeast components; direct demonstration with human HBS1L not shown here
    • How stalled ribosomes are initially distinguished from elongating ones at the A-site not resolved
  2. 2012 Medium

    Extended ribosome rescue to organellar translocons, showing Dom34:Hbs1 clears ribosomes stalled at the ER (Sec61) and mitochondrial (TOM40) import channels.

    Evidence Yeast deletion strains with nonstop reporters targeted to ER or mitochondria; cell fractionation

    PMID:22981232

    Open questions at the time
    • Conducted in yeast; mammalian organellar rescue not directly tested
    • Does not establish physical contact between the complex and translocon components
  3. 2013 Medium

    Demonstrated the mammalian complex requirement and its physical coupling to degradation machinery in non-stop decay.

    Evidence Non-stop reporter degradation assays, Co-IP of Hbs1-Dom34 with Ski2/Mtr4/Dis3, and siRNA knockdown in mammalian cells

    PMID:23667253

    Open questions at the time
    • Co-IP does not define direct vs indirect contacts within the exosome-SKI assembly
    • Isoform-specific contributions not resolved at this stage
  4. 2014 High

    Showed the complex acts on inactive monosomes beyond aberrant transcripts, splitting Stm1-clamped 80S ribosomes with ABCE1 to permit translation restart.

    Evidence In vitro ribosome dissociation assays plus in vivo polysome profiling and glucose-starvation restart assays in yeast

    PMID:24424461

    Open questions at the time
    • Stm1-dependent mechanism described in yeast; mammalian equivalent not addressed
    • Trigger that recruits the complex to inactive ribosomes during stress unclear
  5. 2017 High

    Resolved the isoform logic: a short HBS1L splice product (SKI7/HBS1LV3) handles exosome bridging while the canonical isoform handles PELOTA-dependent rescue, defining division of labor in human cells.

    Evidence Proteomics, reciprocal Co-IP, mRNA half-life measurements, and domain mutant (RxxxFxxxL/RRP43) analysis

    PMID:28204585

    Open questions at the time
    • Regulation of isoform splicing not addressed
    • Structural basis of HBS1LV1 antagonism of supercomplex formation not resolved
  6. 2019 High

    Defined the cellular consequences of HBS1L loss in mammals: monosome accumulation, PELOTA destabilization via the proteasome, and mTOR/translation dysregulation linked to disease.

    Evidence Patient cells with biallelic mutation, polysome profiling, ribosome sequencing, proteasome rescue, and Hbs1l knockdown mouse model

    PMID:30707697

    Open questions at the time
    • Mechanism linking HBS1L loss to mTOR/4-EBP upregulation not defined
    • How PELOTA is targeted for proteasomal degradation in HBS1L absence unknown
  7. 2023 Medium

    Placed HBS1L in the ubiquitin-initiated no-go decay pathway, downstream of ZNF-598 ribosomal ubiquitination and upstream of NONU-1 nuclease cleavage.

    Evidence C. elegans forward/reverse genetics, NGD reporter, ribosomal ubiquitination site mutations, and epistasis of ZNF-598/HBS-1/PELO-1/NONU-1

    PMID:36626369

    Open questions at the time
    • Direct biochemical link between ubiquitinated ribosome and HBS-1 recruitment not shown in this study
    • Pathway order inferred from genetics, not reconstitution
  8. 2023 Medium

    Identified a translation-surveillance-independent application: HBS1L knockdown raises fetal hemoglobin in thalassemic erythroid progenitors, implicating it in globin regulation.

    Evidence Lentiviral shRNA knockdown in primary β0-thalassemia/HbE erythroid progenitors with RT-qPCR, HbF quantification, and flow cytometry

    PMID:36888630

    Open questions at the time
    • Molecular mechanism connecting HBS1L to γ-globin induction unknown
    • Whether effect is via ribosome rescue or another route untested
  9. 2024 Medium

    Established HBS1L ribosome rescue as essential for photoreceptor maintenance, defining the retinal dystrophy phenotype mechanistically.

    Evidence Human patient ERG data, Hbs1l hypomorph mouse, OCT, TUNEL, and mass-spectrometry proteomics

    PMID:38966981

    Open questions at the time
    • Causal chain from rescue failure to photoreceptor apoptosis not dissected
    • Why photoreceptors are selectively vulnerable unexplained
  10. 2025 High

    Revealed a synthetic-lethal dependency with FOCAD and exploited it pharmacologically, showing HBS1L/PELO and FOCAD/SKI cooperate to resolve ribosomal stalling.

    Evidence Combinatorial and genome-wide CRISPR screens with FOCAD isogenic rescue and xenografts; cryo-EM-guided molecular glue degrader (TNG961) with in vitro ubiquitination and in vivo dosing

    PMID:41101730 PMID:42001523

    Open questions at the time
    • Precise molecular interplay between FOCAD/SKI and HBS1L/PELO complexes not fully defined
    • Resistance mechanisms to degrader-induced rescue loss unaddressed
  11. 2026 High

    Provided the structural basis for recruitment: the shared N-terminal UBAh domain binds ubiquitin via the Ile44 patch, explaining how both HBS1L and SKI7 are directed to ubiquitinated stalled ribosomes.

    Evidence NMR structure of mouse UBAh-ubiquitin complex with HSQC titration and Kd measurement

    PMID:42234679

    Open questions at the time
    • Low-affinity interaction (Kd ~50 µM); in vivo requirement of UBAh-ubiquitin binding for rescue not demonstrated here
    • Specificity for particular ubiquitin chain types or ribosomal substrates unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • How HBS1L's molecular surveillance function connects to its tissue-specific physiological roles (fetal hemoglobin regulation, ATF4 reinitiation, neuronal/retinal maintenance) remains mechanistically unresolved.
  • No unified mechanism linking ribosome rescue defects to globin switching
  • ATF4 reinitiation role rests on a preprint and awaits peer-reviewed confirmation
  • Direct demonstration that UBAh-ubiquitin recruitment operates in each physiological context lacking

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003924 GTPase activity 3 GO:0045182 translation regulator activity 3 GO:0060090 molecular adaptor activity 2 GO:0140096 catalytic activity, acting on a protein 2
Localization
GO:0005840 ribosome 3 GO:0005829 cytosol 2
Pathway
R-HSA-8953854 Metabolism of RNA 3 R-HSA-8953897 Cellular responses to stimuli 2
Partners
ABCE1CRBNDIS3EXOSC8FOCADMTREXPELOSKIV2L
Complex memberships
PELOTA-HBS1L ribosome rescue complexSKI complexcytoplasmic RNA exosome (via SKI7/HBS1LV3)

Evidence

Reading pass · 17 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2010 Dom34:Hbs1 (yeast ortholog of PELO:HBS1L) interacts with stalled ribosomes to promote ribosomal subunit dissociation and peptidyl-tRNA drop-off, initiating no-go decay (NGD). This activity is shared with the translation termination factor complex eRF1:eRF3, and is independent of peptide length or A-site codon identity. Reconstituted yeast translation system (in vitro ribosome dissociation and peptidyl-tRNA drop-off assays) Science High 20947765
2010 Hbs1 (yeast) structure was determined with and without GDP, and a low-resolution model of the Dom34-Hbs1 complex was obtained. The complex mimics elongation factor-tRNA or eRF1-eRF3 complexes, supporting binding to the ribosomal A-site. Nucleotide (GTP/GDP) binding by Hbs1 is essential for both NGD and 18S NRD. Dom34-Hbs1 interaction is essential for NGD but largely dispensable for 18S NRD, genetically uncoupling the two pathways. Crystal structure of Hbs1 ± GDP; low-resolution model of Dom34-Hbs1 complex; site-directed mutagenesis of Hbs1 nucleotide-binding and Dom34-interaction surfaces; in vivo NGD and 18S NRD reporter assays Nature structural & molecular biology High 21102444
2013 Mammalian Hbs1L and its binding partner Dom34/PELOTA are required for non-stop mRNA decay (NSD) in mammalian cells. Hbs1L-Dom34 forms a complex with the exosome-SKI complex (Ski2/Mtr4 and Dis3). Elimination of aberrant proteins from non-stop transcripts additionally requires the RING finger E3 ligase listerin. Non-stop mRNA reporter degradation assays in mammalian cells; Co-immunoprecipitation of Hbs1-Dom34 with Ski2/Mtr4/Dis3; siRNA knockdown The Journal of biological chemistry Medium 23667253
2014 The Dom34-Hbs1 complex, together with the Rli1/ABCE1 NTPase, dissociates inactive (non-translating) 80S ribosomes stabilized by the Stm1 clamping factor in glucose-starved yeast, thereby facilitating translation restart upon stress relief. This role in splitting inactive ribosomes occurs both during stress and in growing yeast. In vitro ribosome dissociation assays; in vivo polysome profiling and translation restart assays in yeast; glucose starvation model The EMBO journal High 24424461
2012 Dom34:Hbs1 (yeast) is required to release nonstop proteins stuck in the ER translocon (Sec61 complex) and the mitochondrial translocon (TOM40 complex). By rescuing stalled ribosomes at these organellar translocators, Dom34:Hbs1 clears the channels and restores normal protein import into organelles. Yeast genetic experiments; nonstop mRNA reporter constructs targeting ER or mitochondria; cell fractionation; dom34/hbs1 deletion strains Cell reports Medium 22981232
2017 A short splicing isoform of HBS1L (HBS1LV3/SKI7) is the functional equivalent of yeast Ski7, linking the cytoplasmic exosome and SKI complexes in humans. The canonical isoform HBS1LV1 associates with PELOTA (Dom34) for ribosome rescue but does not bind the exosome. Both isoforms bind the SKI complex, and HBS1LV1 antagonizes SKI/exosome supercomplex formation. The C-terminal RxxxFxxxL motif of HBS1LV3 mediates exosome binding, interacting with exosome core subunit RRP43. Proteomic/mass spectrometry analysis of HBS1L isoform complexes; Co-immunoprecipitation; siRNA depletion; mRNA half-life measurements; transcriptome analysis Nucleic acids research High 28204585
1998 Human eRFS (HBS1L) encodes a GTP-binding protein whose C-terminal domain shares structural features with eEF-1A and eRF3. Phylogenetically, HBS1L and yeast Hbs1p form a cluster branching with the eRF3 family. However, human HBS1L does not complement yeast eRF3/Sup35p thermosensitive mutations and does not interact with eRF1 in yeast — it does not carry eRF3-like translation termination activity. cDNA cloning; phylogenetic analysis; yeast complementation assay; yeast two-hybrid FEBS letters Medium 9872408
2019 Loss of HBS1L in human patient cells causes accumulation of 80S monosomes, increased translation efficiency of ribosomal RNA, upregulation of mTOR and 4-EBP protein expression, and depletion of PELOTA protein at the post-translational level (rescued by proteasome inhibition). An Hbs1l knockdown mouse model recapitulates facial dysmorphism, growth restriction, retinal deposits, and reduced Pelota levels. Patient cell lines with biallelic HBS1L mutation; polysome profiling; Western blot; RT-PCR; ribosome sequencing; proteasome inhibition rescue experiment; mouse Hbs1l knockdown model PLoS genetics High 30707697
2015 The Pelo (Dom34)-Hbs1 mRNA surveillance complex is required for transposon silencing in the Drosophila germline. Pelo functions at the translational level to silence TEs; this function requires interaction with Hbs1. Overexpression of RpS30a (a ribosomal protein) partially rescues TE-silencing defects in pelo mutants. PiRNA biogenesis is not affected by Pelo loss. Drosophila pelo and hbs1 mutant analysis; TE mRNA/protein quantification; genetic epistasis (RpS30a overexpression rescue); piRNA sequencing EMBO reports Medium 26124316
2019 Drosophila Hbs1 is required for spermatogenesis: hbs1 mutant males are sterile due to defects in meiosis and spermatid individualization. Hbs1 genetically interacts with pelota during spermatid individualization, and a point mutation in Pelota's Hbs1-binding site abolishes rescue of spermatogenesis, demonstrating the Pelota-Hbs1 complex is functionally required for this process. Drosophila hbs1 loss-of-function mutants; fertility assays; genetic interaction analysis with pelota hypomorphs; Pelota binding-site point mutant rescue experiment Scientific reports Medium 30824860
2023 In C. elegans, the ribosome rescue factor HBS-1 (HBS1L ortholog) is required for No-Go mRNA Decay (NGD). Ubiquitination of ribosomal proteins eS10 and uS10 is functionally required for NGD. HBS-1 and PELO-1 act downstream of the ubiquitin ligase ZNF-598 and are required for mRNA decay via the nuclease NONU-1. C. elegans forward and reverse genetics; novel NGD reporter; ribosomal ubiquitination site mutations; epistasis analysis of ZNF-598, HBS-1, PELO-1, NONU-1 PLoS genetics Medium 36626369
2024 Loss of HBS1L causes retinal dystrophy in both a human patient and in Hbs1ltm1a/tm1a hypomorph mice, characterized by photoreceptor apoptosis and outer retinal thinning. Proteomic analysis revealed 480 downregulated proteins including rhodopsin, peripherin-2, and EDF1 (a ribosome collision sensor), as well as PELOTA. These findings establish HBS1L's ribosomal rescue function as essential for photoreceptor cell maintenance. Human patient clinical data (ERG); Hbs1ltm1a/tm1a mouse model; OCT imaging; TUNEL assay; mass spectrometry proteomics; GSEA/GO analysis Disease models & mechanisms Medium 38966981
2025 HBS1L is synthetic lethal with FOCAD loss in cancer cells. Mechanistically, HBS1L loss in FOCAD-deleted cells leads to translational arrest and activation of the unfolded protein response (UPR). The FOCAD/SKI complex and HBS1L/PELO complex work together to resolve aberrant mRNA-induced ribosomal stalling. In vivo, HBS1L deletion eliminated growth of FOCAD-deleted tumors. Combinatorial CRISPR screen; genome-wide CRISPR screen in FOCAD isogenic cells; FOCAD re-expression rescue; orthogonal HBS1L manipulation; UPR/translational arrest readouts; xenograft mouse model Biochimica et biophysica acta. Molecular cell research Medium 41101730
2026 TNG961, a molecular glue degrader, promotes HBS1L-CRBN-compound complex formation and induces E3 ligase (cereblon)-dependent HBS1L ubiquitination and degradation. HBS1L degradation disrupts the HBS1L/PELO ribosome-rescue complex, causing translational arrest and UPR activation selectively in FOCAD-negative cancer cells. Cryo-EM structures guided compound optimization. Cryo-EM structural determination of HBS1L-CRBN-compound complex; in vitro ubiquitination assay; proteome selectivity profiling; cell viability assays; xenograft mouse model with oral TNG961 administration Cancer discovery High 42001523
2026 The N-terminal UBAh domain of HBS1L (shared by both HBS1L and SKI7 isoforms) adopts a three-helix bundle architecture and directly binds ubiquitin via its hydrophobic patch interacting with the Ile44-centered hydrophobic patch of ubiquitin (Kd ~50 µM), with a distinctive double β-turn in the α1/α2 loop accommodating the ubiquitin β-turn. The hallmark VLGD/E motif was identified. This interaction suggests HBS1L and SKI7 are recruited to ubiquitinated stalled ribosomes via UBAh. NMR structure of mouse UBAh-ubiquitin complex; HSQC titration experiments; dissociation constant measurement PloS one High 42234679
2025 In Drosophila, loss of Hbs1 (HBS1L ortholog) reduces expression of the stress-responsive transcription factor ATF4, which is encoded by an mRNA with multiple upstream open reading frames (uORFs). In human cells, HBS1L and PELOTA promote translation reinitiation at the ATF4 ORF by facilitating proper translation termination at preceding uORFs. Loss of Hbs1 in Drosophila lamina neurons causes visual defects (ERG abnormalities) and vacuolization/synapse defects; restoring ATF4 in lamina neurons partially rescues ERG defects. Drosophila Hbs1 loss-of-function mutants; ERG assays; confocal microscopy; human cell knockdown; ATF4 translation reporter assays; neuronal-specific rescue experiments bioRxivpreprint Medium 41279977
2023 Knockdown of HBS1L (all known isoforms) using shRNA in erythroid progenitors from β0-thalassemia/HbE patients upregulates γ-globin mRNA (~1.69-fold) and increases fetal hemoglobin percentage (~16.7-fold), with only modest perturbation of red cell differentiation, establishing a direct functional role for HBS1L protein in erythroid globin regulation. Lentiviral shRNA knockdown of HBS1L in primary β0-thalassemia/HbE erythroid progenitors; RT-qPCR for globin mRNAs; HbF quantification; flow cytometry for erythroid differentiation PloS one Medium 36888630

Source papers

Stage 0 corpus · 71 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2008 DNA polymorphisms at the BCL11A, HBS1L-MYB, and beta-globin loci associate with fetal hemoglobin levels and pain crises in sickle cell disease. Proceedings of the National Academy of Sciences of the United States of America 456 18667698
2010 Dom34:Hbs1 promotes subunit dissociation and peptidyl-tRNA drop-off to initiate no-go decay. Science (New York, N.Y.) 273 20947765
2008 Interactions between UPF1, eRFs, PABP and the exon junction complex suggest an integrated model for mammalian NMD pathways. The EMBO journal 261 18256688
2007 Intergenic variants of HBS1L-MYB are responsible for a major quantitative trait locus on chromosome 6q23 influencing fetal hemoglobin levels in adults. Proceedings of the National Academy of Sciences of the United States of America 251 17592125
2014 HBS1L-MYB intergenic variants modulate fetal hemoglobin via long-range MYB enhancers. The Journal of clinical investigation 156 24614105
2015 Genetic variation at MECOM, TERT, JAK2 and HBS1L-MYB predisposes to myeloproliferative neoplasms. Nature communications 146 25849990
2011 A 3-bp deletion in the HBS1L-MYB intergenic region on chromosome 6q23 is associated with HbF expression. Blood 104 21385855
2019 Ethylene Response Factor (ERF) Family Proteins in Abiotic Stresses and CRISPR-Cas9 Genome Editing of ERFs for Multiple Abiotic Stress Tolerance in Crop Plants: A Review. Molecular biotechnology 96 30600447
2003 Eukaryotic release factors (eRFs) history. Biology of the cell 85 12867083
2013 The Hbs1-Dom34 protein complex functions in non-stop mRNA decay in mammalian cells. The Journal of biological chemistry 77 23667253
2009 The HBS1L-MYB intergenic interval associated with elevated HbF levels shows characteristics of a distal regulatory region in erythroid cells. Blood 77 19528534
2014 Association of variants at BCL11A and HBS1L-MYB with hemoglobin F and hospitalization rates among sickle cell patients in Cameroon. PloS one 74 24667352
2007 The HBS1L-MYB intergenic region on chromosome 6q23.3 influences erythrocyte, platelet, and monocyte counts in humans. Blood 72 17712044
2013 A genome-wide screen for ethylene-induced ethylene response factors (ERFs) in hybrid aspen stem identifies ERF genes that modify stem growth and wood properties. The New phytologist 70 23815789
2014 Dom34-Hbs1 mediated dissociation of inactive 80S ribosomes promotes restart of translation after stress. The EMBO journal 69 24424461
2010 Dissection of Dom34-Hbs1 reveals independent functions in two RNA quality control pathways. Nature structural & molecular biology 60 21102444
2012 Roles of dom34:hbs1 in nonstop protein clearance from translocators for normal organelle protein influx. Cell reports 57 22981232
2010 The XmnI (G)gamma polymorphism influences hemoglobin F synthesis contrary to BCL11A and HBS1L-MYB SNPs in a cohort of 57 beta-thalassemia intermedia patients. Blood cells, molecules & diseases 50 20472475
2008 The HBS1L-MYB intergenic region on chromosome 6q23 is a quantitative trait locus controlling fetal haemoglobin level in carriers of beta-thalassaemia. Journal of medical genetics 40 18697826
2017 A short splicing isoform of HBS1L links the cytoplasmic exosome and SKI complexes in humans. Nucleic acids research 39 28204585
1998 The product of the mammalian orthologue of the Saccharomyces cerevisiae HBS1 gene is phylogenetically related to eukaryotic release factor 3 (eRF3) but does not carry eRF3-like activity. FEBS letters 37 9872408
2017 Hypoxia-responsive ERFs involved in postdeastringency softening of persimmon fruit. Plant biotechnology journal 33 28301712
2017 A long noncoding RNA from the HBS1L-MYB intergenic region on chr6q23 regulates human fetal hemoglobin expression. Blood cells, molecules & diseases 33 29227829
2014 DNA polymorphisms at BCL11A, HBS1L-MYB and Xmn1-HBG2 site loci associated with fetal hemoglobin levels in sickle cell anemia patients from Northern Brazil. Blood cells, molecules & diseases 33 25084696
2023 SUMOylation-modified Pelota-Hbs1 RNA surveillance complex restricts the infection of potyvirids in plants. Molecular plant 32 36597359
2013 Disruption of the Hbs1l-Myb locus causes hereditary persistence of fetal hemoglobin in a mouse model. Molecular and cellular biology 32 23428869
2002 Wounding activates immediate early transcription of genes for ERFs in tobacco plants. Plant molecular biology 27 12090623
2016 Modifying effect of XmnI, BCL11A, and HBS1L-MYB on clinical appearances: A study on β-thalassemia and hemoglobin E/β-thalassemia patients in Indonesia. Hematology/oncology and stem cell therapy 26 27009595
2019 Mammalian Hbs1L deficiency causes congenital anomalies and developmental delay associated with Pelota depletion and 80S monosome accumulation. PLoS genetics 25 30707697
2014 Genotyping of BCL11A and HBS1L-MYB SNPs associated with fetal haemoglobin levels: a SNaPshot minisequencing approach. BMC genomics 22 24502199
2020 Functional polymorphisms of BCL11A and HBS1L-MYB genes affect both fetal hemoglobin level and clinical outcomes in a cohort of children with sickle cell anemia. Annals of hematology 19 32447424
2015 The RNA surveillance complex Pelo-Hbs1 is required for transposon silencing in the Drosophila germline. EMBO reports 17 26124316
2008 Association of SNP in exon 1 of HBS1L with hemoglobin F level in beta0-thalassemia/hemoglobin E. International journal of hematology 17 18839276
2012 Influence of BCL11A, HBS1L-MYB, HBBP1 single nucleotide polymorphisms and the HBG2 XmnI polymorphism On Hb F levels. Hemoglobin 16 23094636
2023 Ubiquitination of stalled ribosomes enables mRNA decay via HBS-1 and NONU-1 in vivo. PLoS genetics 14 36626369
2014 Genetic variant in the BCL11A (rs1427407), but not HBS1-MYB (rs6934903) loci associate with fetal hemoglobin levels in Indian sickle cell disease patients. Blood cells, molecules & diseases 14 25457385
2022 Single Nucleotide Polymorphisms in XMN1-HBG2, HBS1L-MYB, and BCL11A and Their Relation to High Fetal Hemoglobin Levels That Alleviate Anemia. Diagnostics (Basel, Switzerland) 13 35741184
2017 Existence of HbF Enhancer Haplotypes at HBS1L-MYB Intergenic Region in Transfusion-Dependent Saudi β-Thalassemia Patients. BioMed research international 13 28280727
2020 The association of HBG2, BCL11A, and HBS1L-MYB polymorphisms to thalidomide response in Chinese β-thalassemia patients. Blood cells, molecules & diseases 12 32387854
1994 The complete sequence of an 18,002 bp segment of Saccharomyces cerevisiae chromosome XI contains the HBS1, MRP-L20 and PRP16 genes, and six new open reading frames. Yeast (Chichester, England) 12 8203164
2021 mRNA surveillance complex PELOTA-HBS1 regulates phosphoinositide-dependent protein kinase1 and plant growth. Plant physiology 11 33930167
2018 A Two-Headed Monster to Avert Disaster: HBS1/SKI7 Is Alternatively Spliced to Build Eukaryotic RNA Surveillance Complexes. Frontiers in plant science 11 30258456
2016 Genetic Variants at BCL11A and HBS1L-MYB loci Influence Hb F Levels in Chinese Zhuang β-Thalassemia Intermedia Patients. Hemoglobin 10 28361591
2019 Pelota-interacting G protein Hbs1 is required for spermatogenesis in Drosophila. Scientific reports 9 30824860
2024 Insect ribosome-rescuer Pelo-Hbs1 complex on sperm surface mediates paternal arbovirus transmission. Nature communications 8 39122673
2021 Modulatory effect of single nucleotide polymorphism in Xmn1, BCL11A and HBS1L-MYB loci on foetal haemoglobin levels in β-thalassemia major and Intermedia patients. JPMA. The Journal of the Pakistan Medical Association 7 34091621
2024 Epigenetic Restriction Factors (eRFs) in Virus Infection. Viruses 6 38399958
2020 Detection of BCL11A and HBS1L-MYB Genotypes in Sickle Cell Anemia. Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion 6 33100714
2024 Low-oxygen-induced root bending is altered by phytoglobin1 through mediation of ethylene response factors (ERFs) and auxin signaling. Planta 5 39012577
2017 Protective BCL11A and HBS1L-MYB polymorphisms in a cohort of 102 Congolese patients suffering from sickle cell anemia. Journal of clinical laboratory analysis 5 28332727
2024 Comprehensive transcriptome analysis of AP2/ERFs in Osmanthus fragrans reveals the role of OfERF017-mediated organic acid metabolism pathway in flower senescence. Frontiers in plant science 4 39391780
2021 Unique Polymorphisms at BCL11A, HBS1L-MYB and HBB Loci Associated with HbF in Kuwaiti Patients with Sickle Cell Disease. Journal of personalized medicine 4 34204365
2022 Fetal hemoglobin-boosting haplotypes of BCL11A gene and HBS1L-MYB intergenic region in the prediction of clinical and hematological outcomes in a cohort of children with sickle cell anemia. Journal of human genetics 3 36167770
2022 Phy meets ERFs to regulate seed germination. Trends in plant science 3 36328871
2022 The progress of protein synthesis factors eIFs, eEFs and eRFs in inflammatory bowel disease and colorectal cancer pathogenesis. Frontiers in oncology 3 36387239
2025 Phenotype of sickle cell disease. Correlation of haplotypes and polymorphisms in cluster β, BCL11A, and HBS1L-MYB. Pilot study. Frontiers in medicine 2 40012971
2025 Genome-wide identification of peanut ERFs and functional characterization of AhERF28 in response to salt and drought stresses. Plant cell reports 2 40593149
2024 HBS1L deficiency causes retinal dystrophy in a child and in a mouse model associated with defective development of photoreceptor cells. Disease models & mechanisms 2 38966981
2026 Comprehensive analysis of AP2/ERFs reveals their functional divergence in safflower flavonoid biosynthesis and stress response. Plant physiology and biochemistry : PPB 1 41547156
2025 Tumor suppressor collateral damage screens reveal mRNA homeostasis protein HBS1L as a novel vulnerability in ch9p21 driven FOCAD deleted cancer. Biochimica et biophysica acta. Molecular cell research 1 41101730
2025 Impact of HBS1L-MYB Gene Single Nucleotide Polymorphisms on Fetal Hemoglobin Expression in Moroccan Sickle Cell Anemia Children: Preliminary Results. Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion 1 41728183
2023 Induction of fetal hemoglobin: Lentiviral shRNA knockdown of HBS1L in β0-thalassemia/HbE erythroid cells. PloS one 1 36888630
2023 Genetic deficiency of ribosomal rescue factor HBS1L causes retinal dystrophy associated with Pelota and EDF1 depletion. bioRxiv : the preprint server for biology 1 37905068
2016 The Impact of XmnI-HBG2, BCL11A and HBS1L-MYB Single Nucleotide Polymorphisms on Hb F Variation of Hematologically Normal Iranian Individuals. Hemoglobin 1 27117569
2026 A genetic risk score based on BCL11A and HBS1L-MYB variants predicts clinical severity in Brazilian sickle cell anaemia patients. British journal of haematology 0 41989145
2026 TNG961 is a selective oral HBS1L molecular glue degrader for the treatment of FOCAD-deleted cancers. Cancer discovery 0 42001523
2026 Solution structure of mouse HBS1L/SKI7-specific UBA domain in complex with ubiquitin: Implications for stalled ribosome recognition. PloS one 0 42234679
2026 Genetic polymorphism of SNPs rs9399137 and rs4895441in HBS1L-MYB and SNP rs766432 in BCL11A among β-thalassemia Egyptian patients. BMC genomic data 0 42237232
2025 Association Between KLF1, BCL11A and HBS1L-MYB Polymorphisms and Phenotypes With β-Thalassemia Patients in Hainan. Molecular genetics & genomic medicine 0 40990146
2025 Translation regulation of ATF4 by the termination complex Hbs1-Pelo is required for visual system development and function. bioRxiv : the preprint server for biology 0 41279977
2024 Associations between BCL11A and HBS1L-MYB polymorphisms and thalassemia risk. Journal of Taibah University Medical Sciences 0 39534793

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