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Showing SECISBP2SBP2 is a alias.

SECISBP2

Selenocysteine insertion sequence-binding protein 2 · UniProt Q96T21

Length
854 aa
Mass
95.5 kDa
Annotated
2026-06-10
38 papers in source corpus 15 papers cited in narrative 15 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

SECISBP2 (SBP2) is the essential, rate-limiting trans-acting factor that directs co-translational incorporation of selenocysteine into selenoproteins, recoding in-frame UGA codons during translation (PMID:10637234, PMID:11118223). It recognizes the SECIS RNA hairpin in selenoprotein mRNA 3' UTRs through an L7A/L30-family RNA-binding domain, contacting the conserved non-Watson-Crick G·A/A·G base-pair quartet and internal loop of the upper helix (PMID:11680849, PMID:12403468). Once bound to a SECIS element, SBP2 recruits the selenocysteine-specific elongation factor eEFSec and does not readily exchange between elements; because SBP2 is limiting and binds different SECIS elements with differing affinity, it establishes the hierarchy of selenoprotein synthesis, favoring mRNAs such as selenoprotein P and PHGPx (PMID:11118223, PMID:17846120). SBP2 also contacts the 60S ribosome at expansion segment ES7L of the 28S rRNA, inducing conformational changes in ES7L-E and the conserved helix H89 (PMID:24850884). Beyond UGA recoding, SBP2 has a separable function in stabilizing selenoprotein mRNAs, as its loss reduces mRNA levels for several selenoproteins without impairing Sec incorporation on remaining transcripts (PMID:27956496). In vivo, SBP2 deficiency causes global selenoprotein synthesis impairment with organ-specific effects on thyroid hormone metabolism via the deiodinases (PMID:29029094). Missense and truncating mutations in SECISBP2 cause a human disorder of abnormal thyroid hormone metabolism through generalized selenoprotein deficiency, with clinical severity modulated by cell-type-specific protein stability and by use of downstream initiation codons that preserve functional domains (PMID:16228000, PMID:19602558, PMID:31350336).

Mechanistic history

Synthesis pass · year-by-year structured walk · 15 steps
  1. 2000 High

    Established that a dedicated SECIS-binding protein is required for selenocysteine insertion, answering whether selenoprotein synthesis needs a specific trans-acting factor beyond the SECIS element itself.

    Evidence UV cross-linking, immunodepletion/recombinant rescue, and in vitro Sec incorporation with 75Se-Sec-tRNA

    PMID:10637234

    Open questions at the time
    • Did not define the SECIS recognition determinants at nucleotide resolution
    • Did not identify downstream factors recruited by SBP2
  2. 2000 High

    Showed how SBP2 acts mechanistically — recruiting eEFSec and acting as the limiting, non-exchanging factor that sets a synthesis hierarchy — answering why some selenoproteins are favored over others.

    Evidence Transfection competition assays comparing overexpressed trans-acting factors and selenoprotein mRNAs

    PMID:11118223

    Open questions at the time
    • Quantitative SECIS affinity differences not measured
    • Did not establish whether hierarchy reflects translation, mRNA stability, or both
  3. 2001 High

    Defined the precise SBP2 footprint on SECIS RNA, answering which structural features of the hairpin are recognized.

    Evidence Enzymatic and hydroxyl radical footprinting, EMSA, and phosphate-ethylation interference

    PMID:11680849

    Open questions at the time
    • No co-crystal or solution structure of the SBP2–SECIS complex
    • Protein residues mediating contacts not yet identified
  4. 2002 High

    Identified the SBP2 RNA-binding domain as L7A/L30-family and pinpointed residues required for SECIS recognition, linking SBP2 to a known RNA-binding fold.

    Evidence Structure-guided alanine scanning informed by the 15.5 kD–U4 snRNA crystal structure, with gel shift validation

    PMID:12403468

    Open questions at the time
    • No direct SBP2 structure determined
    • Contributions of non-RBD regions to function not addressed
  5. 2005 High

    Connected SBP2 to human disease, answering whether SBP2 loss-of-function has physiological consequences, by linking mutations to abnormal thyroid hormone metabolism via generalized selenoprotein deficiency.

    Evidence Genetic linkage, sequencing, and DIO2 enzymatic activity in patient fibroblasts across multiple families

    PMID:16228000

    Open questions at the time
    • Did not resolve why partial loss yields tissue-specific phenotypes
    • Molecular impact of individual mutations not biochemically dissected
  6. 2007 High

    Demonstrated that differential SBP2 binding affinity, not a generic SECIS-binder, dictates the selenoprotein hierarchy in vivo and links it to mRNA translation and NMD sensitivity.

    Evidence siRNA knockdown plus reciprocal Co-IP of endogenous SBP2 with mRNA quantitation

    PMID:17846120

    Open questions at the time
    • Mechanism coupling SBP2 binding to NMD not detailed
    • Quantitative affinity ranking across the full selenoproteome incomplete
  7. 2008 Medium

    Revealed isoform diversity and a mitochondria-targeted variant, answering whether SBP2 function extends beyond cytoplasmic selenoprotein synthesis.

    Evidence Minigene splicing assays, antisense modulation, and mitochondrial localization of mtSBP2

    PMID:19004874

    Open questions at the time
    • Function of mtSBP2 in mitochondria not established
    • Stress-regulated splicing not reconstituted mechanistically
  8. 2009 Medium

    Explained the mild phenotype of a severe truncating mutation through downstream ATG usage producing functional shorter isoforms.

    Evidence Minigene constructs and in vitro translation of R128X mutant

    PMID:19602558

    Open questions at the time
    • Single method, single lab
    • In vivo abundance and activity of downstream-initiated isoforms not quantified
  9. 2010 Medium

    Mapped a disease mutation (R770X) directly to loss of SECIS binding, connecting genotype to a defined biochemical defect.

    Evidence Gel shift assays of mutant proteins and selenoprotein P measurement in compound heterozygotes

    PMID:20501692

    Open questions at the time
    • Single binding method without reciprocal validation
    • Cellular consequences of partial binding loss not fully resolved
  10. 2014 High

    Located the SBP2–ribosome interface at 28S rRNA ES7L, answering how SBP2 physically engages the translation machinery.

    Evidence Bifunctional cross-linking, hydroxyl radical probing, and chemical probing of 28S rRNA

    PMID:24850884

    Open questions at the time
    • High-resolution structure of the SBP2–ribosome complex lacking
    • Functional consequence of induced H89 conformational change unproven
  11. 2017 High

    Separated SBP2's mRNA-stabilizing function from UGA recoding and showed it is not strictly required for Sec incorporation, refining the model of SBP2 essentiality.

    Evidence Ribosome profiling and RNA-seq comparing conditional Secisbp2 vs Trsp knockout mouse livers, with mRNA half-life measurements

    PMID:27956496

    Open questions at the time
    • Molecular mechanism of mRNA stabilization unknown
    • Why effects are gene-specific not explained
  12. 2017 High

    Quantified in vivo organ-specific selenoprotein and deiodinase deficits, linking SBP2 loss to tissue-differential thyroid hormone metabolism.

    Evidence Tamoxifen-inducible conditional knockout mouse with deiodinase activity assays and serum thyroid hormone measurements

    PMID:29029094

    Open questions at the time
    • Basis of organ-specific severity not mechanistically resolved
    • Non-thyroidal selenoprotein consequences not fully characterized
  13. 2019 High

    Showed that cell-type-specific protein stability of mutant SBP2 dictates clinical phenotype, explaining tissue-differential disease severity.

    Evidence Knock-in mouse models of patient mutations with ribosome profiling, thermal stability assays, and tissue protein quantification

    PMID:31350336

    Open questions at the time
    • Degradation pathway controlling tissue-specific stability not identified
    • Generalizability across other mutations untested
  14. 2019 Medium

    Extended SBP2 function to immunometabolism, showing its deficiency in adipose tissue macrophages drives ROS, inflammasome activation, and insulin resistance.

    Evidence ATM-specific siRNA knockdown and re-expression in obese mice with ROS, inflammasome, and insulin sensitivity readouts

    PMID:31453320

    Open questions at the time
    • Which selenoproteins mediate the antioxidant/anti-inflammatory effect not identified
    • Single lab, not independently confirmed
  15. 2025 Medium

    Confirmed SBP2's essential role in human hepatocyte selenoprotein synthesis and distinguished its transcriptomic footprint from paralog SECISBP2L.

    Evidence CRISPR-Cas9 knockout in HepG2 with RNA-seq, mass spectrometry, and immunoblot (preprint)

    PMID:bio_10.1101_2025.07.02.662884

    Open questions at the time
    • Preprint, single lab
    • Functional division of labor between SBP2 and SECISBP2L not mechanistically defined

Open questions

Synthesis pass · forward-looking unresolved questions
  • How SBP2 mechanistically stabilizes selenoprotein mRNAs and the high-resolution architecture of the SBP2–SECIS–eEFSec–ribosome assembly remain unresolved.
  • No structure of SBP2 bound to SECIS or the ribosome
  • mRNA-stabilization mechanism uncharacterized
  • Mitochondrial mtSBP2 function unknown

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 4 GO:0045182 translation regulator activity 3 GO:0060090 molecular adaptor activity 2
Localization
GO:0005739 mitochondrion 1 GO:0005840 ribosome 1
Pathway
R-HSA-392499 Metabolism of proteins 3 R-HSA-8953854 Metabolism of RNA 2
Partners

Evidence

Reading pass · 15 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2000 SBP2 was purified as a SECIS-binding protein and shown to be essential for co-translational selenocysteine insertion. Immunodepletion of SBP2 from cell lysates abolished selenocysteine incorporation into selenoprotein mRNAs in vitro, and this was rescued by adding back recombinant SBP2. The activity was both SBP2- and SECIS element-dependent. UV cross-linking, immunoprecipitation, in vitro Sec incorporation assay with 75Se-labeled Sec-tRNA, immunodepletion and recombinant protein rescue The EMBO journal High 10637234
2000 SBP2 recruits the selenocysteine-specific elongation factor (eEFSec) to selenoprotein mRNA via SECIS binding. Overexpression of SBP2 (but not selenocysteyl-tRNA or eEFSec) overcame competition from excess selenoprotein mRNAs, establishing SBP2 as the limiting trans-acting factor. SBP2, once bound to SECIS elements, does not readily exchange between them. SBP2 preferentially stimulates selenocysteine incorporation from selenoprotein P and PHGPx SECIS elements over others, establishing a hierarchy of selenoprotein synthesis. Transfection-based competition assay, co-expression of trans-acting factors, overexpression of selenoprotein mRNAs The EMBO journal High 11118223
2001 SBP2 binds to a conserved site on SECIS RNA hairpins: it protects the proximal part of the hairpin and 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, the 5' strand of the lower helix, and part of the 5' strand of the internal loop prevented SBP2 binding. Enzymatic and hydroxyl radical footprinting, gel mobility shift analysis, phosphate-ethylation binding interference RNA (New York, N.Y.) High 11680849
2002 SBP2 shares an RNA-binding domain of the L7A/L30 family with the U4 snRNA-binding protein 15.5 kD/Snu13p. Structure-guided alanine scanning of 12 SBP2 residues predicted from alignment with the 15.5 kD–U4 snRNA crystal structure identified four residues whose mutation severely diminished or abolished SECIS RNA binding, with the other eight causing intermediate effects, defining the key amino acids for SECIS recognition. Multiple sequence alignment, structure-guided alanine mutagenesis, gel shift assays RNA (New York, N.Y.) High 12403468
2005 Homozygous and compound heterozygous missense mutations in SECISBP2 in humans cause a global defect in selenoprotein synthesis, resulting in abnormal thyroid hormone metabolism. SBP2 is epistatic to selenoprotein synthesis, so its partial loss has a generalized effect on selenoproteins including deiodinase 2. Genetic linkage analysis, sequencing, fibroblast DIO2 enzymatic activity assay Nature genetics High 16228000
2007 SBP2 exhibits strong preferential binding to some selenoprotein mRNAs over others in vivo (determined by immunoprecipitation and mRNA quantitation), whereas nucleolin exhibits minimal differences in binding. Knockdown of SBP2 confirmed that SBP2 binding affinity is a major determinant dictating the hierarchy of selenoprotein synthesis via differential mRNA translation and sensitivity to nonsense-mediated decay. SBP2 knockdown by siRNA, immunoprecipitation of SBP2 followed by mRNA quantitation, selenoprotein mRNA level measurement Molecular and cellular biology High 17846120
2008 Alternative splicing of SECISBP2 produces at least 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 undergo 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 mitochondrial targeting sequence identification and localization Nucleic acids research Medium 19004874
2009 A nonsense mutation R128X in SBP2 results in synthesis of shorter SBP2 isoforms from at least three downstream ATGs, all of which retain the essential functional domains for SECIS binding. This explains why a severe truncating mutation produces a relatively mild phenotype of partial SBP2 deficiency. Sequencing, minigene construction, in vitro translation analysis of mutant proteins The Journal of clinical endocrinology and metabolism Medium 19602558
2010 The SBP2 truncation mutation R770X (in the RNA-binding domain) inhibits binding of SBP2 to SECIS elements, as shown by gel shift assay, whereas R120X disrupts all functional motifs. Compound heterozygous R120X/R770X causes widespread selenoprotein deficiency including undetectable selenoprotein P. Gel shift assay of mutant SBP2 proteins, sequencing, selenoprotein P measurement The Journal of clinical endocrinology and metabolism Medium 20501692
2014 SBP2 contacts the human ribosome primarily through the 28S rRNA at expansion segment ES7L, specifically helix ES7L-E. SBP2 binding to 80S ribosomes or 60S subunits protects helix ES7L-E from hydroxyl radical cleavage and induces conformational changes in ES7L-E and the universally conserved helix H89 of the 28S rRNA. Cross-linking with bifunctional reagents (diepoxybutane), direct hydroxyl radical probing of 28S rRNA, chemical probing RNA (New York, N.Y.) High 24850884
2017 Ribosome profiling and RNA-seq of conditional Secisbp2 and Trsp (tRNA-Sec) knockout mouse livers showed that Secisbp2 loss results in gene-specific (variable) effects on ribosome density downstream of UGA-Sec codons, distinct from the uniform loss seen with tRNA-Sec depletion. For several selenoproteins, Secisbp2 loss greatly reduced mRNA levels without affecting translational activity or Sec incorporation efficiency on remaining RNA. These data demonstrate that Secisbp2 has a distinct role in stabilizing selenoprotein mRNAs separable from its role in UGA redefinition, and that Secisbp2 is not strictly required for Sec incorporation. Ribosome profiling, RNA-seq, mRNA half-life measurements, conditional genetic knockout (Cre-lox) in mouse liver Nucleic acids research High 27956496
2017 Sbp2 conditional knockout mice have decreased deiodinase 1 expression and enzymatic activity in liver, decreased deiodinase 2 enzymatic activity and deiodinase 3 expression in cerebrum, and decreased expression of other selenoproteins in brain, liver, and serum, demonstrating that SBP2 deficiency causes global selenoprotein synthesis impairment with organ-specific effects on thyroid hormone metabolism. Tamoxifen-inducible conditional knockout mouse model, deiodinase enzymatic activity assays, selenoprotein expression analysis, serum thyroid hormone measurements Endocrinology High 29029094
2019 Pathogenic missense mutation C696R in the RNA-binding domain of SECISBP2 abrogates SECIS binding and does not support selenoprotein translation above the level of a complete null mutation. The R543Q missense mutation in the selenocysteine insertion domain results in residual translational activity but causes thermally unstable protein that is completely degraded in mouse liver in vivo while being partially functional in brain, demonstrating that cell-type-specific protein stability dictates clinical phenotypes. Mouse knock-in models of patient mutations, ribosome profiling, in vitro thermal stability assay, immunoblot for protein levels in different tissues The Journal of biological chemistry High 31350336
2019 SBP2 deficiency in adipose tissue macrophages (ATMs) causes increased intracellular reactive oxygen species and inflammasome activation, leading to IL-1β-driven proinflammatory macrophage expansion. ATM-specific knockdown of SBP2 in obese mice promoted insulin resistance via increased fat tissue inflammation; re-expression of SBP2 improved insulin sensitivity. ATM-specific siRNA knockdown in obese mice, re-expression experiments, ROS and inflammasome measurements, insulin sensitivity assays Science advances Medium 31453320
2025 SBP2 targeting in HepG2 cells (CRISPR-Cas9) impaired selenoprotein mRNA and protein expression, confirming its essential role in selenoprotein synthesis in human hepatocytes, while producing a transcriptomic signature enriched for metabolic and ion transport processes distinct from that of its paralog SECISBP2L. CRISPR-Cas9 gene editing, RNA-seq, mass spectrometry, immunoblot bioRxivpreprint Medium bio_10.1101_2025.07.02.662884

Source papers

Stage 0 corpus · 38 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 303 10637234
2005 Mutations in SECISBP2 result in abnormal thyroid hormone metabolism. Nature genetics 288 16228000
2020 K2 Sb(P2 O7 )F: Cairo Pentagonal Layer with Bifunctional Genes Reveal Optical Performance. Angewandte Chemie (International ed. in English) 135 32745331
2000 SECIS-SBP2 interactions dictate selenocysteine incorporation efficiency and selenoprotein hierarchy. The EMBO journal 133 11118223
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 29 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 13 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 11 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
2026 A Rare Cause of Thyroid Hormone Abnormalities in an Adolescent: A Case Report of SBP2 (SECISBP2) Deficiency. Molecular syndromology 0 42238688
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

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