Affinage

SRP54

Signal recognition particle subunit SRP54 · UniProt P61011

Length
504 aa
Mass
55.7 kDa
Annotated
2026-04-28
50 papers in source corpus 21 papers cited in narrative 21 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SRP54 is the universally conserved GTPase subunit of the signal recognition particle (SRP) that couples nascent-chain signal-sequence recognition to co-translational protein targeting at the endoplasmic reticulum or thylakoid membrane. Its methionine-rich M domain binds hydrophobic signal sequences and anchors SRP54 to SRP RNA—in mammals dependent on prior SRP19 binding to helix 8—while the NG GTPase domain undergoes a cooperative nucleotide cycle with the SRP receptor (SRα/FtsY): both proteins start nucleotide-free, co-bind GTP to form the targeting complex that transfers the nascent chain to the Sec61 translocon, and hydrolyze GTP to dissociate (PMID:9182758, PMID:7969124, PMID:9016569). De novo missense mutations in the SRP54 GTPase domain cause Shwachman–Diamond-like syndrome with severe congenital neutropenia; these mutations destabilize the GTPase core, abolish GTP binding and receptor-complex formation, trigger ER stress and p53-dependent apoptosis in granulocytic progenitors, and act dominant-negatively by impairing XBP1 mRNA splicing (PMID:28972538, PMID:33053321, PMID:29914977, PMID:33227812). In chloroplasts, cpSRP54 additionally functions posttranslationally—forming a transit complex with cpSRP43 via its plastid-specific C-terminal tail—to chaperone light-harvesting proteins (LHCP) to the thylakoid membrane (PMID:7731984, PMID:25833951, PMID:38989593).

Mechanistic history

Synthesis pass · year-by-year structured walk · 15 steps
  1. 1994 High

    Establishing that GTPase activity of SRP54 is essential for protein export in vivo, and that GTP-locked mutants exert dominant-negative effects, defined the enzymatic cycle as a functional requirement rather than a passive binding event.

    Evidence Site-directed mutagenesis of S. pombe Srp54 with in vivo growth assays, GTPase assays, and precursor accumulation assays

    PMID:7969124

    Open questions at the time
    • Mechanism of dominant-negative inhibition at the molecular level unclear
    • No structural data on mutant conformations
  2. 1995 High

    Discovery that chloroplast SRP54 functions as a molecular chaperone for posttranslational LHCP integration into thylakoids revealed an SRP-independent, RNA-free targeting mode for this conserved GTPase.

    Evidence In vitro reconstitution of transit complex formation, antibody depletion and complementation assays

    PMID:7731984

    Open questions at the time
    • How cpSRP54 discriminates LHCP from other substrates not resolved
    • No structural data on the transit complex
  3. 1997 High

    Defining the complete nucleotide cycle—empty-site start, cooperative GTP binding to both SRP54 and SRα, and coupled hydrolysis for complex dissociation—established the mechanistic framework for signal-sequence transfer to Sec61.

    Evidence In vitro reconstitution with GTP analogs, GTPase activity and complex assembly/dissociation biochemical assays

    PMID:9182758

    Open questions at the time
    • Structural basis of cooperative GTP binding unknown at this point
    • Mechanism of signal sequence handoff to translocon not defined
  4. 1997 High

    Mapping the SRP19-dependent SRP54M binding site to SRP RNA helix 8 explained why eukaryotic SRP assembly requires an ordered pathway and identified the RNA determinants of M-domain docking.

    Evidence Systematic site-directed mutagenesis of SRP RNA, in vitro binding assays with purified SRP54M and SRP19

    PMID:11680843 PMID:9016569

    Open questions at the time
    • Conformational change induced by SRP19 not structurally resolved
    • Whether SRP19 contacts SRP54 directly or acts solely through RNA unknown
  5. 2000 High

    The first crystal structure of the SRP54 NG domain confirmed the conserved GTPase fold and validated, by Thr112Ala mutagenesis, that an active-site threonine is catalytically essential, providing an atomic framework for understanding disease mutations.

    Evidence X-ray crystallography at 2.0 Å of archaeal Ffh NG domain plus active-site mutagenesis

    PMID:10801496

    Open questions at the time
    • No full-length structure showing M-domain orientation relative to NG domain
    • No structure of the SRP54–SRα heterodimer
  6. 2002 High

    Comprehensive alanine scanning of the M domain identified discrete residues required for SRP RNA binding and showed that RNA binding remodels the signal-peptide groove and monomer/dimer equilibrium, linking RNA association to substrate recognition competence.

    Evidence Systematic tri-alanine mutagenesis (40 substitutions) with in vitro RNA binding and gel filtration

    PMID:12234178

    Open questions at the time
    • No direct structural visualization of groove remodeling upon RNA binding
    • Whether dimer-to-monomer transition is physiologically relevant unclear
  7. 2008 High

    The full-length GDP-bound SRP54 crystal structure revealed an articulated linker connecting NG and M domains, explaining how signal-sequence binding in the M domain could allosterically communicate with the GTPase active site.

    Evidence X-ray crystallography at 2.5 Å of full-length Pyrococcus furiosus SRP54

    PMID:18953414

    Open questions at the time
    • No structure in GTP-bound or receptor-complexed state
    • Linker dynamics not directly measured
  8. 2008 Medium

    Identification of two amino acid substitutions in the cpSRP54 RNA-binding domain that abolish SRP RNA binding explained the evolutionary loss of SRP RNA from the chloroplast SRP complex in land plants.

    Evidence In vitro RNA binding assays, site-directed mutagenesis, phylogenetic analysis

    PMID:18755190

    Open questions at the time
    • Whether these substitutions were the initial evolutionary events or secondary is unknown
    • Functional consequences of RNA loss for targeting efficiency not quantified
  9. 2015 Medium

    Mapping the cpSRP54–cpSRP43 heterodimer interface to the cpSRP54 C-terminal tail and cpSRP43 chromodomain 2 explained how land-plant chloroplasts acquired an RNA-independent posttranslational targeting complex absent in algae.

    Evidence Co-immunoprecipitation, domain mutagenesis, comparative biochemistry between algae and land plants

    PMID:25833951

    Open questions at the time
    • Structural basis of the cpSRP54–cpSRP43 interaction not determined
    • Evolutionary intermediates between algal and land-plant systems unknown
  10. 2017 High

    Discovery that de novo SRP54 GTPase-domain mutations cause Shwachman–Diamond-like syndrome with congenital neutropenia established SRP54 as a disease gene and linked impaired GTP hydrolysis to failed granulocyte development.

    Evidence GTPase activity assays on purified mutant proteins, SRP54 knockdown zebrafish model, clinical genetics

    PMID:28972538

    Open questions at the time
    • How granulocytes are selectively vulnerable among cell types not explained
    • Downstream secretory substrates whose loss causes neutropenia not identified
  11. 2018 High

    Demonstrating that SRP54 deficiency triggers p53-dependent apoptosis and ER stress with autophagy markers in granulocytic cells identified the cellular death pathway downstream of SRP54 loss and connected it to the unfolded protein response.

    Evidence SRP54 knockdown cell lines, primary patient bone marrow cells, apoptosis and ER stress marker analysis

    PMID:29914977

    Open questions at the time
    • Whether p53 activation is direct or secondary to ER stress not resolved
    • Contribution of autophagy to cell death versus survival not dissected
  12. 2020 High

    Structural characterization of three disease-causing SRP54 variants showed that mutations destabilize the entire GTPase core, abolish GTP binding, and prevent SRP–SR targeting complex formation, providing a unified structural explanation for loss of function.

    Evidence X-ray crystallography, HDX-MS, biochemical GTPase assays, yeast and human cell-based targeting complex formation

    PMID:33053321

    Open questions at the time
    • Whether partial GTPase activity remains in heterozygous patients not quantified
    • No structure of mutant SRP54 in complex with SRP RNA
  13. 2021 High

    Epistasis experiments in zebrafish placed SRP54 upstream of XBP1 splicing and showed dominant-negative pathogenesis: mutant SRP54 aggravated neutropenia in heterozygous fish, and spliced XBP1 rescued the phenotype, identifying the IRE1–XBP1 axis as the critical downstream effector.

    Evidence Zebrafish srp54+/− with mutant mRNA injection, xbp1 mRNA rescue, granulocytic differentiation in HL-60 and CD34+ HSPCs

    PMID:33227812

    Open questions at the time
    • Mechanism by which SRP54 mutations impair XBP1 splicing not defined
    • Whether the XBP1 connection is granulocyte-specific or generalizable unknown
  14. 2024 Medium

    In planta dissection showed the cpSRP54 GTPase is required for both co- and post-translational chloroplast targeting pathways, while its C-terminal tail is exclusively needed for the posttranslational LHCP route, functionally separating the two targeting modes.

    Evidence Arabidopsis cpSRP54 knockout complementation with truncation and GTPase point-mutation variants

    PMID:38989593

    Open questions at the time
    • Structural basis for C-terminal tail selectivity not resolved
    • How GTPase cycle operates in cotranslational chloroplast targeting with reduced SRP RNA not clear
  15. 2025 Medium

    Zebrafish srp54 null mutants revealed a cell-type-selective developmental requirement: motor axon outgrowth and branching are specifically impaired, extending SRP54's in vivo roles beyond hematopoiesis to neuronal development.

    Evidence Zebrafish srp54 nonsense mutant, motor axon imaging, motility assay, cell-type-specific analysis

    PMID:40328346

    Open questions at the time
    • Which secretory substrates are critical for motor axon development not identified
    • Whether motor neuron vulnerability reflects high secretory demand or a specific SRP54-dependent cargo unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include: why specific cell types (granulocytes, motor neurons, exocrine pancreas) are selectively vulnerable to SRP54 deficiency; the identity of the critical cargo proteins whose mistargeting drives each tissue phenotype; and the molecular mechanism linking SRP54 mutations to impaired IRE1–XBP1 signaling.
  • Cargo specificity of SRP54 in granulocytes not mapped
  • Structural basis for dominant-negative mechanism in heterozygotes not determined
  • No reconstitution of the SRP54→IRE1→XBP1 axis with purified components

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003924 GTPase activity 6 GO:0003723 RNA binding 3 GO:0044183 protein folding chaperone 1
Localization
GO:0009536 plastid 4 GO:0005783 endoplasmic reticulum 2 GO:0009579 thylakoid 2 GO:0005829 cytosol 1
Pathway
R-HSA-1643685 Disease 4 R-HSA-9609507 Protein localization 4 R-HSA-8953854 Metabolism of RNA 3 R-HSA-392499 Metabolism of proteins 2 R-HSA-5357801 Programmed Cell Death 1
Partners
CPSRP43SEC61A1SRP19SRPRASRPRBTP53XBP1
Complex memberships
Signal recognition particle (SRP)cpSRP (cpSRP54–cpSRP43 heterodimer)

Evidence

Reading pass · 21 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1997 SRP54 and SRα GTPases are in the empty (nucleotide-free) site conformation prior to contact between the SRP-ribosome complex and the membrane-bound SRP receptor. Cooperative GTP binding to both SRP54 and SRα stabilizes the SRP-SR complex and initiates signal sequence transfer from SRP54 to Sec61α. GTP hydrolysis by both SRP54 and SRα is required for dissociation of the SRP-SR complex. In vitro reconstitution with GTP analogs, biochemical assays of GTPase activity and complex assembly/dissociation Cell High 9182758
1994 The S. pombe Srp54 GTPase activity is essential for protein export; GTPase-deficient mutations (R194L/H, T248N) confer lethal or conditional phenotypes, and dominant-negative GTP-locked mutants inhibit wild-type Srp54 function, whereas a GDP-locked mutant does not interfere with wild-type protein. Site-directed mutagenesis, in vivo growth assays, immunoprecipitation-based GTPase assay, precursor accumulation assay Molecular and cellular biology High 7969124
1997 Human SRP54 binding to SRP RNA requires prior binding of protein SRP19; site-directed mutagenesis of SRP RNA helix 8 identified the primary binding site for the M-domain of SRP54 (SRP54M), and SRP19 is essential for formation of the ternary SRP19/SRP54M/SRP RNA complex in the human system. Systematic site-directed mutagenesis of SRP RNA, in vitro protein-RNA binding assays with purified SRP54M and SRP19 Nucleic acids research High 9016569
2000 Crystal structure of the archaeal SRP54/Ffh GTPase (NG) domain from Acidianus ambivalens at 2.0 Å revealed a conserved GTPase fold; mutation of Thr112→Ala abolishes GTP hydrolysis; structural comparison supports a heterodimeric SRP-SR interaction model for signal transduction. X-ray crystallography, active-site mutagenesis (Thr112Ala), structural comparison with bacterial and eukaryotic homologs Structure High 10801496
2008 Crystal structure of full-length GDP-bound SRP54 from Pyrococcus furiosus at 2.5 Å showed complete domain organization: a Ras-like GTPase domain with tightly bound GDP, a flexible α-helical linker acting as an articulated arm, and an M domain for signal peptide scanning; the linker is structurally coupled to the GTPase catalytic site and likely propagates conformational changes upon signal sequence binding. X-ray crystallography (2.5 Å resolution), structural analysis PloS one High 18953414
2020 X-ray crystallography and hydrogen-deuterium exchange mass spectrometry of three disease-causing SRP54 variants (T115A, T117Δ, G226E) revealed extensive structural destabilization of the GTPase core, abolition of GTP binding, and elimination of SRP receptor (SRα/FtsY) targeting complex formation in both yeast and human cells. X-ray crystallography, HDX-MS, biochemical GTPase assays, yeast and human cell-based targeting complex formation assays Structure High 33053321
2021 SRP54 mutations cause congenital neutropenia via dominant-negative impairment of unconventional XBP1 mRNA splicing; injection of mutant SRP54 mRNAs into srp54+/- zebrafish aggravated neutropenia, and rescue was achieved by spliced (active) but not unspliced xbp1 mRNA, placing SRP54 upstream of XBP1 in the pathway. Zebrafish knockout/morphant model, mRNA rescue experiments, epistasis with xbp1 morphants, granulocytic differentiation assays in HL-60 cells and CD34+ HSPCs Blood High 33227812
2017 De novo missense mutations in SRP54 affecting the GTPase domain impair GTPase activity; the mutated proteins show reduced GTP hydrolysis in vitro, and SRP54 knockdown in zebrafish causes neutropenia and reduced exocrine pancreas size, recapitulating the human Shwachman-Diamond-like phenotype. GTPase activity assays on purified mutant proteins, SRP54 knockdown zebrafish model, 3D protein modeling The Journal of clinical investigation High 28972538
2018 SRP54 mutations or knockdown lead to drastically reduced proliferation of granulocytic cells associated with enhanced p53-dependent apoptosis, and bone marrow examination reveals dysgranulopoiesis with cellular ER stress and autophagy markers, confirmed in SRP54-mutated primary cells and SRP54-knockdown cells. SRP54 knockdown cell lines, primary patient cells, bone marrow examination, apoptosis assays, ER stress marker analysis Blood High 29914977
2002 Systematic tri-alanine mutagenesis of the M-domain of human SRP54 identified residues at positions 379–387, 394–396, 400–405, 409–411 as required for SRP RNA binding; mutations at 328-TLR-330 also abolished binding despite being distant from the predicted RNA binding site, and binding to SRP RNA alters the SRP54M monomer/dimer equilibrium and the shape of the signal peptide binding groove. Systematic site-directed mutagenesis (40 alanine substitutions), in vitro RNA binding assays, gel filtration Biochemistry High 12234178
2019 Chloroplast SRP54 (cpSRP54) directly contacts the ribosomal subunit uL4 (not the peptide tunnel exit site) via its plastid-specific C-terminal tail region, enabling early cotranslational membrane targeting of PsbA (D1) before the nascent chain emerges from the ribosome. Ribosome profiling, analysis of membrane-associated vs. soluble ribosome footprints, cpSRP54/ribosome binding interface mapping, truncation analysis of C-terminal tail The Plant cell High 31444312
2020 SRP54 interacts directly with both RIG-I and MDA5 (RIG-I-like receptors) and impairs their association with the adaptor protein VISA (MAVS), thereby negatively regulating IFN-β production and antiviral signaling. Co-immunoprecipitation, overexpression and knockdown assays for IFN-β induction, viral replication assays Virologica Sinica Medium 32767210
2006 SRp54 (SFRS11) acts as a splicing repressor of tau exon 10 by binding a purine-rich element in exon 10 and antagonizing the SR protein Tra2β; overexpression of SRp54 suppresses exon 10 inclusion while RNAi knockdown increases inclusion. GFP reporter for tau exon 10 splicing, expression cloning, RNA interference knockdown, RNA binding assays, deletion mutagenesis Molecular and cellular biology Medium 16943417
1998 SRp54 binds to C-rich pyrimidine tracts between the 5' splice site and branch point of small introns lacking a classical 3' pyrimidine tract, and together with U2AF mediates intron bridging as an alternative early spliceosome assembly mode. RNA binding assays, UV cross-linking, splicing assays with mutant introns, U1/U2 snRNP binding analysis Molecular and cellular biology Medium 9710626
1995 The chloroplast SRP54 homologue (54CP) is essential for transit complex formation with the light-harvesting chlorophyll a/b protein (LHCP) and is required for posttranslational integration of LHCP into the thylakoid membrane, functioning as a molecular chaperone. In vitro reconstitution of transit complex, antibody depletion, import and integration assays Proceedings of the National Academy of Sciences of the United States of America High 7731984
1997 Chloroplast SRP54 (54CP) discriminates between thylakoid-targeting signals based on hydrophobicity, cross-linking to LHCP, cytochrome f, and Rieske FeS protein but not to the 23- and 33-kDa OEC proteins, demonstrating it recognizes a specific subset of precursors. Nascent chain cross-linking, in vitro targeting signal recognition assays The Journal of biological chemistry High 9111079
2015 In higher plant chloroplasts, cpSRP54 forms a stable heterodimer with the chloroplast-specific cpSRP43 required for posttranslational LHCP transport; interaction requires specific residues in the cpSRP54 C-terminal tail and the second chromodomain of cpSRP43, which are absent in the alga Chlamydomonas reinhardtii where the two proteins do not interact. Co-immunoprecipitation, domain mutagenesis, comparative biochemistry between algae and land plants The Journal of biological chemistry Medium 25833951
2008 Two amino acid substitutions within the RNA-binding domain of cpSRP54 in higher plants abolish SRP RNA binding, explaining the absence of SRP RNA from the chloroplast SRP complex in land plants. In vitro RNA binding assays, site-directed mutagenesis, phylogenetic analysis FEBS letters Medium 18755190
2024 The GTPase activity of cpSRP54 is essential for both posttranslational (nuclear-encoded LHCP) and cotranslational (plastid-encoded subunit) transport pathways; the C-terminal tail region of cpSRP54 is required exclusively for posttranslational LHCP transport and cpSRP43 interaction, while its absence causes accumulation of a photosystem I assembly intermediate. Arabidopsis cpSRP54 knockout complementation with truncation and GTPase point-mutation variants, phenotypic analysis Journal of experimental botany Medium 38989593
2001 Assembly control for SRP54M binding to human SRP RNA is localized to a region encompassing RNA residues 177–221 in helix 8; chimeric human/M. jannaschii SRP RNAs showed that helix 8 (not helix 6) conveys SRP19 dependency for SRP54M binding. Chimeric RNA construction, in vitro protein-RNA binding assays with purified SRP19 and SRP54M RNA Medium 11680843
2025 srp54-/- zebrafish exhibit reduced caudal primary motor axon length and branching and reduced motility at 30 hpf, indicating a specific requirement for Srp54 in motor axon development; the hatching gland (a secretory cell type) is also affected, while other cell types examined are not. Zebrafish srp54 nonsense mutant (loss-of-function), motor axon imaging, motility assay, cell-type-specific analysis Neuroscience Medium 40328346

Source papers

Stage 0 corpus · 50 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1995 A chloroplast homologue of the signal recognition particle subunit SRP54 is involved in the posttranslational integration of a protein into thylakoid membranes. Proceedings of the National Academy of Sciences of the United States of America 159 7731984
2017 Mutations in signal recognition particle SRP54 cause syndromic neutropenia with Shwachman-Diamond-like features. The Journal of clinical investigation 124 28972538
1997 Empty site forms of the SRP54 and SR alpha GTPases mediate targeting of ribosome-nascent chain complexes to the endoplasmic reticulum. Cell 109 9182758
2018 Mutations in the SRP54 gene cause severe congenital neutropenia as well as Shwachman-Diamond-like syndrome. Blood 89 29914977
2008 Quantitative proteomics of a chloroplast SRP54 sorting mutant and its genetic interactions with CLPC1 in Arabidopsis. Plant physiology 63 18633119
1997 Chloroplast SRP54 interacts with a specific subset of thylakoid precursor proteins. The Journal of biological chemistry 57 9111079
2006 SRp54 (SFRS11), a regulator for tau exon 10 alternative splicing identified by an expression cloning strategy. Molecular and cellular biology 50 16943417
2000 The crystal structure of the conserved GTPase of SRP54 from the archaeon Acidianus ambivalens and its comparison with related structures suggests a model for the SRP-SRP receptor complex. Structure (London, England : 1993) 49 10801496
1992 A Mycoplasma protein homologous to mammalian SRP54 recognizes a highly conserved domain of SRP RNA. Nucleic acids research 41 1280809
2002 RNA interference of signal peptide-binding protein SRP54 elicits deleterious effects and protein sorting defects in trypanosomes. The Journal of biological chemistry 36 12244113
2019 Ribosome-Associated Chloroplast SRP54 Enables Efficient Cotranslational Membrane Insertion of Key Photosynthetic Proteins. The Plant cell 33 31444312
1996 Identification of a region of Bacillus subtilis Ffh, a homologue of mammalian SRP54 protein, that is essential for binding to small cytoplasmic RNA. The Journal of biological chemistry 33 8662730
1998 A role for SRp54 during intron bridging of small introns with pyrimidine tracts upstream of the branch point. Molecular and cellular biology 31 9710626
1997 Binding site of the M-domain of human protein SRP54 determined by systematic site-directed mutagenesis of signal recognition particle RNA. Nucleic acids research 28 9016569
1994 The Srp54 GTPase is essential for protein export in the fission yeast Schizosaccharomyces pombe. Molecular and cellular biology 28 7969124
2015 Chloroplast SRP54 Was Recruited for Posttranslational Protein Transport via Complex Formation with Chloroplast SRP43 during Land Plant Evolution. The Journal of biological chemistry 27 25833951
1994 The Bacillus subtilis SRP54 homologue, Ffh, has an intrinsic GTPase activity and forms a ribonucleoprotein complex with small cytoplasmic RNA in vivo. Biochemical and biophysical research communications 27 7511896
2021 SRP54 mutations induce congenital neutropenia via dominant-negative effects on XBP1 splicing. Blood 26 33227812
1994 A structure for the signal sequence binding protein SRP54: 3D reconstruction from STEM images of single molecules. Journal of structural biology 26 7880651
2008 Structures of SRP54 and SRP19, the two proteins that organize the ribonucleic core of the signal recognition particle from Pyrococcus furiosus. PloS one 23 18953414
1999 Enhancing effect of Bacillus subtilis Ffh, a homologue of the SRP54 subunit of the mammalian signal recognition particle, on the binding of SecA to precursors of secretory proteins in vitro. Journal of biochemistry 23 9880811
1993 Identification of RNA sequences and structural elements required for assembly of fission yeast SRP54 protein with signal recognition particle RNA. Molecular and cellular biology 23 8382769
2001 An archaeal protein homologous to mammalian SRP54 and bacterial Ffh recognizes a highly conserved region of SRP RNA. FEBS letters 22 11696367
1998 Protein SRP54 of human signal recognition particle: cloning, expression, and comparative analysis of functional sites. Gene 18 9511762
1996 Bacillus subtilis Ffh, a homologue of mammalian SRP54, can intrinsically bind to the precursors of secretory proteins. Biochemical and biophysical research communications 18 8886007
2020 Structural and Functional Impact of SRP54 Mutations Causing Severe Congenital Neutropenia. Structure (London, England : 1993) 17 33053321
2008 Evolutionary substitution of two amino acids in chloroplast SRP54 of higher plants cause its inability to bind SRP RNA. FEBS letters 16 18755190
1997 Yarrowia lipolytica SRP54 homolog and translocation of Kar2p. Yeast (Chichester, England) 12 9178502
2023 Chloroplast SRP43 and SRP54 independently promote thermostability and membrane binding of light-dependent protochlorophyllide oxidoreductases. The Plant journal : for cell and molecular biology 10 37269173
2020 Congenital neutropenia with variable clinical presentation in novel mutation of the SRP54 gene. Pediatric blood & cancer 10 32277798
1995 Modulation of the signal recognition particle 54-kDa subunit (SRP54) in rat preosteoblasts by the extracellular matrix. The Journal of biological chemistry 9 7673110
1994 Structural and functional characterisation of the signal recognition particle-specific 54 kDa protein (SRP54) of tomato. Molecular & general genetics : MGG 9 7808407
2020 SRP54 Negatively Regulates IFN-Beta Production and Antiviral Response by Targeting RIG-I and MDA5. Virologica Sinica 8 32767210
2002 Systematic site-directed mutagenesis of human protein SRP54: interactions with signal recognition particle RNA and modes of signal peptide recognition. Biochemistry 8 12234178
1994 Arabidopsis thaliana expresses three divergent Srp54 genes. Plant physiology 8 7824644
2023 Chloroplast SRP54 and FtsH protease coordinate thylakoid membrane-associated proteostasis in Arabidopsis. Plant physiology 7 36994815
2001 Assembly of the human signal recognition particle (SRP): overlap of regions required for binding of protein SRP54 and assembly control. RNA (New York, N.Y.) 7 11680843
2022 Acute myeloid leukemia in SRP54-mutated congenital neutropenia. EJHaem 6 35846055
2017 The Exon Junction Complex and Srp54 Contribute to Hedgehog Signaling via ci RNA Splicing in Drosophila melanogaster. Genetics 5 28637711
2022 Case Report: Association between cyclic neutropenia and SRP54 deficiency. Frontiers in immunology 4 36159802
2011 Compositional and structural features related to thermal stability in the archaea SRP19 and SRP54 signal recognition particle proteins. Journal of molecular evolution 4 21505884
2004 Two strategically placed base pairs in helix 8 of mammalian signal recognition particle RNA are crucial for the SPR19-dependent binding of protein SRP54. RNA (New York, N.Y.) 3 15037766
2024 The role of chloroplast SRP54 domains and its C-terminal tail region in post- and co-translational protein transport in vivo. Journal of experimental botany 2 38989593
2021 Cellular Traffic Jam and Disease Due to Mutations in SRP54. Structure (London, England : 1993) 2 33417891
2021 Archaeal SRP RNA and SRP19 facilitate the assembly of SRP54-FtsY targeting complex. Biochemical and biophysical research communications 2 34116357
1999 Systematic mutagenesis of the fission yeast Srp54 protein. Current genetics 2 10079327
2002 Identification and characterization of Streptococcus pneumoniae Ffh, a homologue of SRP54 subunit of mammalian signal recognition particle. Biochemical and biophysical research communications 1 11922609
2026 SRP54-related congenital neutropenia: a multidisciplinary effort. BMJ case reports 0 41638760
2025 srp54 promotes motor neuron development and is required for motility in zebrafish. Neuroscience 0 40328346
2024 SRP54 of black carp negatively regulates MDA5-mediated antiviral innate immunity. Developmental and comparative immunology 0 39173725