SRP54
Signal recognition particle subunit SRP54
Also known as: SRP54_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- P61011
- Gene
- SRP54
- Ensembl
- ENSG00000100883
- Chromosome
- 14
- Canonical length
- 504 aa
- Protein class
- Disease related genes, Human disease related genes, Plasma proteins, Potential drug targets, Predicted intracellular proteins, Transporters
- Subcellular location
- Cytosol
OverviewNCBI Gene
Enables several functions, including 7S RNA binding activity; endoplasmic reticulum signal peptide binding activity; and guanyl ribonucleotide binding activity. Contributes to GTPase activity. Involved in granulocyte differentiation and protein targeting to ER. Located in cytosol and nucleus. Part of signal recognition particle, endoplasmic reticulum targeting. Implicated in severe congenital neutropenia 8. [provided by Alliance of Genome Resources, Jul 2025]
Canonical amino-acid sequenceUniProt
504 residues, UniProt reviewed canonical sequence.
>P61011|SRP54
1 MVLADLGRKI TSALRSLSNA TIINEEVLNA MLKEVCTALL EADVNIKLVK QLRENVKSAI
61 DLEEMASGLN KRKMIQHAVF KELVKLVDPG VKAWTPTKGK QNVIMFVGLQ GSGKTTTCSK
121 LAYYYQRKGW KTCLICADTF RAGAFDQLKQ NATKARIPFY GSYTEMDPVI IASEGVEKFK
181 NENFEIIIVD TSGRHKQEDS LFEEMLQVAN AIQPDNIVYV MDASIGQACE AQAKAFKDKV
241 DVASVIVTKL DGHAKGGGAL SAVAATKSPI IFIGTGEHID DFEPFKTQPF ISKLLGMGDI
301 EGLIDKVNEL KLDDNEALIE KLKHGQFTLR DMYEQFQNIM KMGPFSQILG MIPGFGTDFM
361 SKGNEQESMA RLKKLMTIMD SMNDQELDST DGAKVFSKQP GRIQRVARGS GVSTRDVQEL
421 LTQYTKFAQM VKKMGGIKGL FKGGDMSKNV SQSQMAKLNQ QMAKMMDPRV LHHMGGMAGL
481 QSMMRQFQQG AAGNMKGMMG FNNMLocalizationUniProt · AlphaFold · HPA
Whether an antibody against SRP54 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Intracellular
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.31
- Highest tissue expression
- 58 nTPM
Expression across tissuesHPA
Tissue
- liver: 58 nTPM
- salivary gland: 57 nTPM
- pancreas: 49 nTPM
- testis: 41 nTPM
- tonsil: 40 nTPM
- parathyroid gland: 37 nTPM
Single-cell type
- late spermatids: 1,633 nCPM
- early spermatids: 375 nCPM
- plasma cells: 296 nCPM
- neutrophils: 295 nCPM
- pancreatic acinar cells: 256 nCPM
- late primary spermatocytes: 202 nCPM
Immune cell
- T-reg: 48 nTPM
- non-classical monocyte: 47 nTPM
- NK-cell: 43 nTPM
- MAIT T-cell: 40 nTPM
- eosinophil: 39 nTPM
- intermediate monocyte: 35 nTPM
Brain region
- white matter: 23 nTPM
- hypothalamus: 23 nTPM
- choroid plexus: 22 nTPM
- pons: 21 nTPM
- spinal cord: 20 nTPM
- medulla oblongata: 20 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about SRP54.
Disease | AllUniProt
Conditions SRP54 is implicated in, by any mechanism.
- Neutropenia, severe congenital 8, autosomal dominant (SCN8) MIM:618752
Disease | GeneticClinVar
7 pathogenic / likely-pathogenic of 352 ClinVar records.
Conditions with pathogenic or likely-pathogenic variants.
- Neutropenia, severe congenital, 8, autosomal dominant
- Shwachman-Diamond syndrome 1
- Inborn genetic diseases
- Ciliary dyskinesia, primary, 40
Disease | AutoantibodyPubMed
Conditions in which antibodies against SRP54 are reported. Each links to that disease's full target list.
- Myositis 68
- Dermatomyositis 14
- Polymyositis 14
- Lung Diseases, Interstitial 9
- Muscle Weakness 9
- Arthritis 3
- Scleroderma, Systemic 3
Showing 7 of 10 — disease pages carrying at least 10 antigens.
ReferencesPubMed · IEDB
Publications for SRP54 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
121 publications
- Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy.
2016 · J Neurol Neurosurg Psychiatry · RCR 9.9 · 231 citations - The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome.
2014 · Autoimmun Rev · RCR 9.2 · 227 citations - Autoantibody profiles in the sera of European patients with myositis.
2001 · Ann Rheum Dis · RCR 8.2 · 305 citations - Clinical Features and Treatment Outcomes of Necrotizing Autoimmune Myopathy.
2015 · JAMA Neurol · RCR 7.9 · 180 citations - Thigh muscle MRI in immune-mediated necrotising myopathy: extensive oedema, early muscle damage and role of anti-SRP autoantibodies as a marker of severity.
2017 · Ann Rheum Dis · RCR 7.9 · 146 citations
Show 20 more of 121 total
- Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy.
2006 · Ann Rheum Dis · RCR 7.3 · 261 citations - Inflammatory myopathy with anti-signal recognition particle antibodies: case series of 100 patients.
2015 · Orphanet J Rare Dis · RCR 6.9 · 167 citations - Case study of CD19 CAR T therapy in a subject with immune-mediate necrotizing myopathy treated in the RESET-Myositis phase I/II trial.
2024 · Mol Ther · RCR 6.3 · 42 citations - Longitudinal Course of Disease in a Large Cohort of Myositis Patients With Autoantibodies Recognizing the Signal Recognition Particle.
2017 · Arthritis Care Res (Hoboken) · RCR 6.1 · 118 citations - Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series.
2010 · Arthritis Care Res (Hoboken) · RCR 5.9 · 178 citations - Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy.
2004 · Arthritis Rheum · RCR 5.9 · 223 citations - Zilucoplan in immune-mediated necrotising myopathy: a phase 2, randomised, double-blind, placebo-controlled, multicentre trial.
2023 · Lancet Rheumatol · RCR 5.9 · 42 citations - Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy.
2011 · Arthritis Rheum · RCR 5.3 · 168 citations - Treatment of refractory immune-mediated necrotizing myopathy with efgartigimod.
2024 · Front Immunol · RCR 4.3 · 21 citations - Anti-SRP immune-mediated necrotizing myopathy: A critical review of current concepts.
2022 · Front Immunol · RCR 3.8 · 39 citations - Seronegative patients form a distinctive subgroup of immune-mediated necrotizing myopathy.
2019 · Neurol Neuroimmunol Neuroinflamm · RCR 3.7 · 66 citations - Emerging atypical clinicopathological manifestations of immune-mediated necrotizing myopathy (IMNM).
2025 · Neuromuscul Disord · RCR 3.1 · 8 citations - Distinct seasonal patterns in the onset of adult idiopathic inflammatory myopathy in patients with anti-Jo-1 and anti-signal recognition particle autoantibodies.
1991 · Arthritis Rheum · RCR 3.1 · 96 citations - Comprehensive Enteroviral Serology Links Infection and Anti-Melanoma Differentiation-Associated Protein 5 Dermatomyositis.
2025 · ACR Open Rheumatol · RCR 3 · 8 citations - Myopathy associated with antibodies to signal recognition particle: disease progression and neurological outcome.
2012 · Arch Neurol · RCR 2.7 · 79 citations - Interstitial lung disease is not rare in immune-mediated necrotizing myopathy with anti-signal recognition particle antibodies.
2022 · BMC Pulm Med · RCR 2.6 · 25 citations - Myositis-specific autoantibodies in Japanese patients with juvenile idiopathic inflammatory myopathies.
2019 · Mod Rheumatol · RCR 2.4 · 38 citations - Myositis-specific and myositis-associated autoantibodies in Indian patients with inflammatory myositis.
2016 · Rheumatol Int · RCR 2.1 · 40 citations - Therapeutic plasma exchange for the treatment of refractory necrotizing autoimmune myopathy.
2022 · J Clin Apher · RCR 2.1 · 18 citations - Performance evaluation of a commercial line blot assay system for detection of myositis- and systemic sclerosis-related autoantibodies.
2020 · Clin Rheumatol · RCR 2 · 29 citations
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 0.22
- gnomAD pLI
- 1
- gnomAD missense Z
- 3.45
- DepMap mean gene effect
- -2.26
- DepMap dependency class
- pan
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 7% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
- exocrine pancreas development
- granulocyte differentiation
- neutrophil chemotaxis
- protein targeting to ER
- SRP-dependent cotranslational protein targeting to membrane
- SRP-dependent cotranslational protein targeting to membrane, signal sequence recognition
- SRP-dependent cotranslational protein targeting to membrane, translocation
Molecular functions
- 7S RNA binding
- endoplasmic reticulum signal peptide binding
- GDP binding
- GTP binding
- GTPase activity
- ribonucleoprotein complex binding
- RNA binding
Cellular components
Protein domainsUniProt · Pfam · InterPro
- Signal recognition particle, SRP54 subunit, GTPase domain
- AAA+ ATPase domain
- Signal recognition particle SRP54, helical bundle
- P-loop containing nucleoside triphosphate hydrolase
- SRP/SRP receptor, N-terminal
- Signal recognition particle SRP54, N-terminal domain superfamily
- SRP54-type protein, GTPase domain
- SRP54-type protein, helical bundle domain
- Signal recognition particle, SRP54 subunit, M-domain
- Signal recognition particle, SRP54 subunit, eukaryotic
- Signal recognition particle, SRP54 subunit
- Signal recognition particle, SRP54 subunit, M-domain superfamily
- Signal peptide binding domain
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of SRP54 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads SRP54 as an antibody target. Whether an autoantibody or antibody against SRP54 could matter depends on whether native SRP54 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
SRP54 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.
Annotation status
The present source text does not explicitly label SRP54 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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