Affinage

SECISBP2

Selenocysteine insertion sequence-binding protein 2 · UniProt Q96T21

Length
854 aa
Mass
95.5 kDa
Annotated
2026-04-28
37 papers in source corpus 14 papers cited in narrative 14 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SECISBP2 (SBP2) is an essential trans-acting factor for selenoprotein synthesis that binds SECIS RNA hairpin elements in the 3′ UTRs of selenoprotein mRNAs and recruits the selenocysteine incorporation machinery to the ribosome. SBP2 recognizes the non-Watson–Crick G·A/A·G base-pair quartet of the SECIS element through its L7Ae/L30-family RNA-binding domain and contacts the 60S ribosomal subunit at helix ES7L-E of the 28S rRNA, inducing conformational changes in universally conserved helix H89 (PMID:10637234, PMID:11680849, PMID:12403468, PMID:24850884). Beyond facilitating UGA-to-selenocysteine recoding, SBP2 independently stabilizes selenoprotein mRNAs against nonsense-mediated decay in a gene-specific, hierarchical manner, with differential SECIS-binding affinity determining which selenoproteins are preferentially synthesized under limiting SBP2 conditions (PMID:17846120, PMID:27956496). Homozygous or compound heterozygous loss-of-function mutations in SECISBP2 cause a multisystem selenoprotein deficiency syndrome characterized by abnormal thyroid hormone metabolism, and tissue-specific phenotypic severity is dictated by differential SBP2 protein stability across cell types (PMID:16228000, PMID:31350336).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2000 High

    Identifying SBP2 as the essential SECIS-binding factor resolved how the selenocysteine incorporation machinery is recruited to selenoprotein mRNAs during translation.

    Evidence UV cross-linking, immunodepletion/add-back reconstitution in rabbit reticulocyte lysate in vitro translation system

    PMID:10637234

    Open questions at the time
    • Binding site on SECIS RNA not yet mapped at nucleotide resolution
    • Ribosome contact sites unknown
    • Mechanism of selenocysteine elongation factor recruitment not addressed
  2. 2000 High

    Demonstrating that SBP2 does not readily exchange between SECIS elements and preferentially stimulates certain selenoproteins established the concept of a hierarchy in selenoprotein synthesis governed by SBP2 availability.

    Evidence Transfection-based competition assay with multiple SECIS elements and SBP2 co-expression

    PMID:11118223

    Open questions at the time
    • Molecular basis of differential SECIS affinity not defined
    • In vivo hierarchy not yet confirmed
  3. 2001 High

    Nucleotide-resolution mapping of the SBP2 footprint on SECIS RNA pinpointed the non-Watson–Crick G·A/A·G quartet as the critical recognition element, defining the RNA determinants of binding specificity.

    Evidence Enzymatic and hydroxyl radical footprinting, phosphate-ethylation interference, gel shift assays

    PMID:11680849

    Open questions at the time
    • Structural basis of recognition not resolved at atomic level
    • How quartet differences among SECIS elements produce differential SBP2 affinity unclear
  4. 2002 High

    Identifying SBP2's RNA-binding domain as an L7Ae/L30 family fold and mapping critical residues by alanine scanning established the protein-side determinants of SECIS recognition.

    Evidence Structure-guided mutagenesis with gel shift assays for SECIS binding

    PMID:12403468

    Open questions at the time
    • No co-crystal structure of SBP2-SECIS complex
    • How the same fold discriminates SECIS from other kink-turn RNAs not explained
  5. 2005 High

    Discovery of human SECISBP2 mutations causing generalized selenoprotein deficiency proved that SBP2 is epistatic to the entire selenoproteome in vivo and linked it to a Mendelian syndrome of abnormal thyroid hormone metabolism.

    Evidence Genetic linkage analysis, SECISBP2 sequencing, fibroblast DIO2 activity assay, serum selenoprotein measurements in affected individuals

    PMID:16228000

    Open questions at the time
    • Genotype-phenotype correlations across tissues not established
    • Whether residual selenoprotein synthesis reflects alternative Sec insertion pathways unclear
  6. 2007 High

    In vivo RIP-qPCR and SBP2 knockdown showed that SBP2 binds selenoprotein mRNAs with widely varying affinity and that limiting SBP2 exposes some mRNAs to nonsense-mediated decay, confirming the selenoprotein synthesis hierarchy in mammalian cells.

    Evidence siRNA knockdown of SBP2, RIP-qPCR for bound selenoprotein mRNAs, mRNA level quantification

    PMID:17846120

    Open questions at the time
    • Whether mRNA stabilization and Sec insertion are mechanistically coupled or fully separable not resolved
    • SECIS features determining NMD sensitivity unknown
  7. 2008 Medium

    Discovery of alternative splicing producing a mitochondrially targeted SBP2 isoform (mtSBP2) raised the possibility of compartmentalized selenoprotein synthesis regulation.

    Evidence Minigene splicing assay, fluorescence microscopy showing mitochondrial localization of mtSBP2

    PMID:19004874

    Open questions at the time
    • Functional role of mtSBP2 in mitochondria not demonstrated
    • Whether mitochondrial selenoprotein synthesis requires SBP2 not tested
    • Findings from a single lab
  8. 2009 Medium

    Internal translation initiation from downstream ATGs in an early-truncation mutant allele explained how severe N-terminal mutations can produce mild phenotypes, revealing that functional SBP2 domains reside in the C-terminal half.

    Evidence Minigene construction and in vitro translation identifying downstream ATG-initiated isoforms

    PMID:19602558

    Open questions at the time
    • In vivo contribution of each downstream ATG-initiated isoform not quantified
    • Single lab, single mutation context
  9. 2014 High

    Mapping SBP2's ribosome contact to expansion segment helix ES7L-E of the 28S rRNA, and showing it induces conformational changes in helix H89, revealed how SBP2 physically connects SECIS-bound mRNA to the ribosomal A-site where Sec-tRNA is delivered.

    Evidence Bifunctional cross-linking, hydroxyl radical probing, and chemical probing of 28S rRNA in SBP2-ribosome complexes

    PMID:24850884

    Open questions at the time
    • No cryo-EM or crystal structure of the SBP2-ribosome complex
    • How H89 conformational change facilitates Sec-tRNA accommodation not mechanistically resolved
  10. 2017 High

    Genetic epistasis comparing Secisbp2 and tRNASec conditional knockouts by ribosome profiling formally demonstrated that SBP2 has two separable functions — facilitating Sec incorporation and stabilizing selenoprotein mRNAs — with gene-specific effects on each.

    Evidence Ribosome profiling and RNA-Seq in conditional knockout mouse livers (Secisbp2 vs. Trsp), mRNA half-life measurements

    PMID:27956496

    Open questions at the time
    • Molecular mechanism of mRNA stabilization (e.g., which decay pathway is inhibited) not identified
    • Whether SBP2 directly shields mRNA or acts through NMD factor exclusion not resolved
  11. 2019 High

    Mouse knockin models of two pathogenic mutations showed that disruption of the RNA-binding domain (C696R) abolishes all function while disruption of the selenocysteine insertion domain (R543Q) produces a thermally unstable protein whose tissue-specific degradation rate dictates tissue-specific phenotype severity.

    Evidence Mouse knockin alleles, ribosome profiling, in vitro thermal stability assays, Western blots across tissues

    PMID:31350336

    Open questions at the time
    • Proteostatic machinery responsible for differential SBP2 degradation across tissues not identified
    • Whether pharmacological stabilization of R543Q could rescue selenoprotein synthesis not tested

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include the atomic structure of the SBP2–SECIS–ribosome ternary complex, the molecular mechanism by which SBP2 stabilizes selenoprotein mRNAs independently of Sec insertion, and the functional significance of the mitochondrially targeted SBP2 isoform.
  • No high-resolution structure of SBP2 bound to SECIS RNA or ribosome
  • Mechanism of mRNA stabilization (NMD shielding vs. direct decay pathway inhibition) unresolved
  • Functional role of mtSBP2 in mitochondria untested

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 4 GO:0045182 translation regulator activity 4
Localization
GO:0005829 cytosol 2 GO:0005739 mitochondrion 1
Pathway
R-HSA-392499 Metabolism of proteins 5 R-HSA-8953854 Metabolism of RNA 2

Evidence

Reading pass · 14 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2000 SBP2 was purified and identified as a novel SECIS RNA-binding protein essential for co-translational selenocysteine incorporation. Immunodepletion of SBP2 from cell lysates abolished selenocysteine insertion, which was restored by adding recombinant SBP2, establishing its essential role in Sec incorporation in vitro. UV cross-linking, immunoprecipitation, in vitro translation with 75Se-labeled Sec-tRNA, immunodepletion and add-back reconstitution The EMBO journal High 10637234
2000 SBP2 overexpression overcomes competition for selenoprotein synthesis caused by excess selenoprotein mRNA, and SBP2 once bound to SECIS elements does not readily exchange between them. SBP2 preferentially stimulates selenocysteine incorporation directed by selenoprotein P and PHGPx SECIS elements over those of other selenoproteins, establishing its role in determining a hierarchy of selenoprotein synthesis. Transfection-based competition assay, co-expression of SBP2 and selenoprotein mRNAs in cells The EMBO journal High 11118223
2001 SBP2 binds the proximal part of SECIS hairpin, protecting both strands of the lower half of the upper helix containing the non-Watson-Crick G·A/A·G base-pair quartet; the G·A/A·G tandem and internal loop are critical for SBP2 binding. Phosphate modification along both strands of the non-Watson-Crick base-pair quartet prevents SBP2 binding. Enzymatic and hydroxyl radical footprinting, gel mobility shift assay, phosphate-ethylation binding interference RNA (New York, N.Y.) High 11680849
2002 SBP2 and the U4 snRNA-binding protein 15.5 kD/Snu13p share the same L7A/L30 family RNA binding domain. Structure-guided alanine substitution of 12 predicted residues identified four whose mutation severely diminished or abolished SECIS RNA binding, mapping critical amino acids for SECIS recognition by SBP2. Multiple sequence alignment, structure-guided mutagenesis, alanine substitution, gel shift assays RNA (New York, N.Y.) High 12403468
2007 SBP2 exhibits strong preferential binding to some selenoprotein mRNAs over others in vivo, and knockdown of SBP2 expression leads to differential effects on selenoprotein mRNA levels and sensitivity to nonsense-mediated decay, establishing SBP2 as a major determinant of the hierarchy of selenoprotein synthesis. siRNA knockdown of SBP2, immunoprecipitation of SBP2 followed by quantitative RT-PCR of bound mRNAs Molecular and cellular biology High 17846120
2008 Alternative splicing of human SECISBP2 produces at least eight splice variants encoding five isoforms with varying N-terminal sequences. One isoform, mtSBP2, contains a mitochondrial targeting sequence and localizes to mitochondria. Full-length SBP2 and some splice variants are subject to coordinated transcriptional and translational regulation in response to UVA irradiation-induced stress. In silico analysis, minigene-based in vivo splicing assay, antisense oligonucleotide modulation, subcellular localization by fluorescence microscopy, UVA stress experiments Nucleic acids research Medium 19004874
2014 SBP2 contacts the human ribosome primarily through the 28S rRNA at helix ES7L-E in expansion segment 7 of the 60S subunit. SBP2 binding to ribosomes induces conformational changes in ES7L-E and the universally conserved helix H89 of 28S rRNA. Bifunctional reagent cross-linking, hydroxyl radical probing of 28S rRNA, diepoxybutane cross-linking, chemical probing RNA (New York, N.Y.) High 24850884
2017 Ribosome profiling and RNA-Seq in mouse liver with conditional deletion of Secisbp2 or Trsp (tRNASec) revealed that Secisbp2 has two separable functions: facilitating Sec incorporation at UGA codons and stabilizing selenoprotein mRNAs. Loss of tRNASec uniformly abolished ribosome density downstream of UGA-Sec codons, while loss of Secisbp2 produced gene-specific variable effects. For several selenoproteins, Secisbp2 loss reduced mRNA levels without affecting translational activity on remaining mRNA, demonstrating a distinct mRNA stability role. Ribosome profiling, RNA-Seq, mRNA half-life measurements, conditional knockout mouse models (Secisbp2 and Trsp), genetic epistasis comparison Nucleic acids research High 27956496
2019 Two pathogenic missense mutations in Secisbp2 were functionally dissected in mouse models: C696R in the RNA-binding domain abrogates SECIS binding and does not support selenoprotein translation above the null level; R543Q in the selenocysteine insertion domain causes residual activity but is thermally unstable in vitro and completely degraded in mouse liver while being partially functional in the brain, demonstrating that differential protein stability in individual cell types dictates tissue-specific phenotypes. Mouse knockin models with pathogenic mutations, ribosome profiling, in vitro thermal stability assay, Western blotting The Journal of biological chemistry High 31350336
2005 Homozygous or compound heterozygous missense mutations in SECISBP2 in humans cause decreased DIO2 enzymatic activity and generalized selenoprotein deficiency, establishing SBP2 as epistatic to selenoprotein synthesis in vivo. The phenotype includes abnormal thyroid hormone metabolism with reduced T3, elevated T4, and reduced glutathione peroxidase and selenoprotein P levels. Genetic linkage analysis, fibroblast DIO2 enzyme activity assay, sequencing of SECISBP2 gene, in vivo human studies Nature genetics High 16228000
2010 The R770X truncation mutation in SBP2 specifically inhibits its binding to SECIS elements in vitro, as demonstrated by gel shift assay, while R120X disrupts all functional motifs. This establishes the C-terminal region (around residue 770) as required for SECIS RNA binding. Gel shift assay with mutant SBP2 proteins, DNA sequencing The Journal of clinical endocrinology and metabolism Medium 20501692
2009 The R128X nonsense mutation in SBP2 allows synthesis of SBP2 isoforms from at least three downstream ATGs that contain all essential functional domains, explaining the relatively mild phenotype caused by an early stop codon. This demonstrates that internal translation initiation can produce functional SBP2 isoforms. Minigene construction, in vitro translation analysis, clinical phenotyping The Journal of clinical endocrinology and metabolism Medium 19602558
2019 SBP2 deficiency in adipose tissue macrophages (ATMs) promotes metabolic activation, increases intracellular reactive oxygen species and inflammasome activity, and promotes IL-1β-associated local proliferation and infiltration of proinflammatory macrophages. ATM-specific SBP2 knockdown in obese mice promoted insulin resistance, while reexpression of SBP2 improved insulin sensitivity. ATM-specific knockdown in vivo in obese mice, ROS measurement, inflammasome assays, metabolic phenotyping Science advances Medium 31453320
2025 CRISPR-Cas9 knockout of SBP2 in HepG2 cells impairs selenoprotein mRNA and protein expression with a transcriptomic signature enriched for metabolic and ion transport processes, distinct from that of the paralog SECISBP2L. SBP2 targeting confirmed its canonical role in selenoprotein synthesis while demonstrating it does not regulate extracellular matrix or cell adhesion pathways (those are SECISBP2L-specific). CRISPR-Cas9 knockout, RNA-seq, mass spectrometry, immunoblotting bioRxivpreprint Medium bio_10.1101_2025.07.02.662884

Source papers

Stage 0 corpus · 37 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2000 A novel RNA binding protein, SBP2, is required for the translation of mammalian selenoprotein mRNAs. The EMBO journal 302 10637234
2005 Mutations in SECISBP2 result in abnormal thyroid hormone metabolism. Nature genetics 288 16228000
2000 SECIS-SBP2 interactions dictate selenocysteine incorporation efficiency and selenoprotein hierarchy. The EMBO journal 133 11118223
2020 K2 Sb(P2 O7 )F: Cairo Pentagonal Layer with Bifunctional Genes Reveal Optical Performance. Angewandte Chemie (International ed. in English) 132 32745331
2001 The selenocysteine incorporation machinery: interactions between the SECIS RNA and the SECIS-binding protein SBP2. RNA (New York, N.Y.) 92 11680849
2007 SBP2 binding affinity is a major determinant in differential selenoprotein mRNA translation and sensitivity to nonsense-mediated decay. Molecular and cellular biology 87 17846120
2009 Clinical and molecular characterization of a novel selenocysteine insertion sequence-binding protein 2 (SBP2) gene mutation (R128X). The Journal of clinical endocrinology and metabolism 82 19602558
2010 Selenoprotein-related disease in a young girl caused by nonsense mutations in the SBP2 gene. The Journal of clinical endocrinology and metabolism 73 20501692
2002 The SBP2 and 15.5 kD/Snu13p proteins share the same RNA binding domain: identification of SBP2 amino acids important to SECIS RNA binding. RNA (New York, N.Y.) 55 12403468
2012 Novel compound heterozygous mutations in the SBP2 gene: characteristic clinical manifestations and the implications of GH and triiodothyronine in longitudinal bone growth and maturation. European journal of endocrinology 50 22247018
2009 Selenium supplementation fails to correct the selenoprotein synthesis defect in subjects with SBP2 gene mutations. Thyroid : official journal of the American Thyroid Association 48 19265499
2017 The RNA-binding protein Secisbp2 differentially modulates UGA codon reassignment and RNA decay. Nucleic acids research 46 27956496
2018 The hsa-miR-181a-5p reduces oxidation resistance by controlling SECISBP2 in osteoarthritis. BMC musculoskeletal disorders 34 30286747
2010 The syndrome of inherited partial SBP2 deficiency in humans. Antioxidants & redox signaling 34 19769464
2008 Functional characterization of alternatively spliced human SECISBP2 transcript variants. Nucleic acids research 31 19004874
2015 Enhancement of lipid peroxidation and its amelioration by vitamin E in a subject with mutations in the SBP2 gene. Journal of lipid research 30 26411970
2016 SBP2 plays an important role in the virulence changes of different artificial mutants of Streptococcus suis. Molecular bioSystems 28 27077729
2018 A Novel Homozygous Selenocysteine Insertion Sequence Binding Protein 2 (SECISBP2, SBP2) Gene Mutation in a Turkish Boy. Thyroid : official journal of the American Thyroid Association 23 29882503
2014 The SBP2 protein central to selenoprotein synthesis contacts the human ribosome at expansion segment 7L of the 28S rRNA. RNA (New York, N.Y.) 21 24850884
2019 SBP2 deficiency in adipose tissue macrophages drives insulin resistance in obesity. Science advances 20 31453320
2019 Ribosome profiling of selenoproteins in vivo reveals consequences of pathogenic Secisbp2 missense mutations. The Journal of biological chemistry 17 31350336
2017 Thyroid Hormone Metabolism Defects in a Mouse Model of SBP2 Deficiency. Endocrinology 17 29029094
2014 Substrate binding protein SBP2 of a putative ABC transporter as a novel vaccine antigen of Moraxella catarrhalis. Infection and immunity 15 24914218
2020 Clinical and Molecular Analysis in 2 Families With Novel Compound Heterozygous SBP2 (SECISBP2) Mutations. The Journal of clinical endocrinology and metabolism 14 32084277
2018 Selenium-sensitive miRNA-181a-5p targeting SBP2 regulates selenoproteins expression in cartilage. Journal of cellular and molecular medicine 14 30247797
2015 The Vaccine Candidate Substrate Binding Protein SBP2 Plays a Key Role in Arginine Uptake, Which Is Required for Growth of Moraxella catarrhalis. Infection and immunity 13 26597985
2014 The truncated major pilin subunit Sbp2 of the srtBCD pilus cluster still contributes to Streptococcus suis pathogenesis in the absence of pilus shaft. Current microbiology 12 24989484
2003 The soybean sucrose binding protein gene family: genomic organization, gene copy number and tissue-specific expression of the SBP2 promoter. Journal of experimental botany 12 14585823
2021 Evaluation of the immunogenicity and protective ability of a pili subunit, SBP2', of Streptococcus suis serotype 2. Research in veterinary science 10 34020335
2006 Combinatorial regulation modules on GmSBP2 promoter: a distal cis-regulatory domain confines the SBP2 promoter activity to the vascular tissue in vegetative organs. Biochimica et biophysica acta 9 16574256
2024 Severe neurodevelopmental phenotype, diagnostic, and treatment challenges in patients with SECISBP2 deficiency. Genetics in medicine : official journal of the American College of Medical Genetics 8 39315526
2007 Distinct repressing modules on the distal region of the SBP2 promoter contribute to its vascular tissue-specific expression in different vegetative organs. Plant molecular biology 8 17710554
2020 Role of the Thyroid Gland in Expression of the Thyroid Phenotype of Sbp2-Deficient Mice. Endocrinology 5 31826256
2025 Case Report: A homozygous selenocysteine insertion sequence-binding protein 2 (SECISBP2) gene mutation in a pediatric patient. Frontiers in pediatrics 1 40918659
2024 The First-Ever Investigation of SNP rs119461977 in SECISBP2/SBP2 Gene and its Implications for Hypothyroidism: A Novel Case-Control Research. Indian journal of clinical biochemistry : IJCB 1 40123624
2025 Biophysical analysis of SECIS binding protein 2 (SBP2) from Naegleria gruberi. Biochimica et biophysica acta. Proteins and proteomics 0 40294688
2025 Functional characterization of promoter regions in selenoprotein synthesis-relevant genes (sbp2, eefsec and sepsecs) and their selenium-dependent regulation in yellow catfish Pelteobagrus fulvidraco. Biochimica et biophysica acta. Gene regulatory mechanisms 0 40618995