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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SRP19
signal recognition particle 19
Chromosome 5 · 5q22.2
NCBI Gene: 6728Ensembl: ENSG00000153037.15HGNC: HGNC:11300UniProt: A0A087WYR0
81PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingsignal recognition particle, endoplasmic reticulum targetingsignal recognition particle7S RNA bindingfamilial adenomatous polyposis 1severe congenital neutropeniaautosomal dominant severe congenital neutropeniaShwachman-Diamond syndrome 1
✦AI Summary

SRP19 is a critical component of the signal recognition particle (SRP), a ribonucleoprotein complex that mediates cotranslational targeting of secretory and membrane proteins to the endoplasmic reticulum 1. The protein directly binds to 7SL RNA and plays an essential scaffolding role in SRP assembly by bridging the tips of RNA helices 6 and 8, creating a stable network of protein-RNA interactions 2. SRP19 binding precedes and promotes the association of SRP54, the main signal sequence recognition component, demonstrating its fundamental role in SRP assembly 2. Structurally, SRP19 exhibits anti-cooperative binding with the SRP68/72 heterodimer, suggesting complex regulatory mechanisms in SRP formation 3. The protein is highly conserved across species, with functional complementation observed between plant and mammalian components 4. Disease relevance includes severe congenital neutropenia caused by SRP19 genetic defects, which disrupt neutrophil differentiation through altered protein processing and ER homeostasis 1. Additionally, SRP19 represents a therapeutic vulnerability in colorectal cancers with APC deletions, where SRP19 is frequently co-deleted, leading to reduced protein secretion capacity and increased ER stress susceptibility 5. The essential nature of SRP19 is demonstrated by its requirement for cell viability in multiple organisms 6.

Sources cited
1
SRP19 mediates cotranslational targeting and causes severe congenital neutropenia when defective
PMID: 36223592
2
SRP19 bridges RNA helices 6 and 8 and promotes SRP54 binding during SRP assembly
PMID: 12050674
3
SRP19 exhibits anti-cooperative binding with SRP68/72 heterodimer
PMID: 18564060
4
SRP19 shows functional conservation between plant and mammalian systems
PMID: 9225861
5
SRP19 represents a therapeutic vulnerability in APC-deleted colorectal cancers
PMID: 40208946
6
SRP19 is essential for cell viability and protein secretion
PMID: 9426009
Disease Associationsⓘ20
familial adenomatous polyposis 1Open Targets
0.42Moderate
severe congenital neutropeniaOpen Targets
0.38Weak
autosomal dominant severe congenital neutropeniaOpen Targets
0.37Weak
Shwachman-Diamond syndrome 1Open Targets
0.33Weak
neurodegenerative diseaseOpen Targets
0.28Weak
ArthropathyOpen Targets
0.15Weak
aortic valve stenosisOpen Targets
0.03Suggestive
cancerOpen Targets
0.01Suggestive
dengue diseaseOpen Targets
0.01Suggestive
constitutional neutropeniaOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
Merkel cell skin cancerOpen Targets
0.01Suggestive
neutropeniaOpen Targets
0.01Suggestive
systemic lupus erythematosusOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.00Suggestive
breast cancerOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
infectionOpen Targets
0.00Suggestive
preeclampsiaOpen Targets
0.00Suggestive
familial pancreatic carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants1
NM_003135.3(SRP19):c.189+5G>APathogenic
Shwachman-Diamond syndrome 1;Severe congenital neutropenia
☆☆☆☆2020
View on ClinVar ↗
Related Genes
RPL12Protein interaction100%RPL13AProtein interaction100%RPL38Protein interaction100%RPL27Protein interaction100%RPL29Protein interaction100%RPLP0Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
90%
Brain
88%
Heart
59%
Lung
48%
Ovary
36%
Gene Interaction Network
Click a node to explore
SRP19RPL12RPL13ARPL38RPL27RPL29RPLP0
PROTEIN STRUCTURE
Preparing viewer…
PDB1JID · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.79LoF Tolerant
pLIⓘ
0.07Tolerant
Observed/Expected LoF0.48 [0.30–0.79]
RankingsWhere SRP19 stands among ~20K protein-coding genes
  • #5,903of 20,598
    Most Researched81
  • #4,961of 5,498
    Most Pathogenic Variants1
  • #6,498of 17,882
    Most Constrained (LOEUF)0.79
Genes detectedSRP19
Sources retrieved10 papers
Response time—
📄 Sources
10â–¼
1
Human genetic defects in SRP19 and SRPRA cause severe congenital neutropenia with distinctive proteome changes.
PMID: 36223592
Blood · 2023
1.00
2
PMID: 40208946
Proc Natl Acad Sci U S A · 2025
0.90
3
Interaction of rice and human SRP19 polypeptides with signal recognition particle RNA.
PMID: 9225861
Plant Mol Biol · 1997
0.80
4
Isolation and cloning of the Yarrowia lipolytica SEC65 gene, a component of the yeast signal recognition particle displaying homology with the human SRP19 gene.
PMID: 9426009
Gene · 1997
0.70
5
Comparative Analysis of Gene Expression Profiles of Human Dental Fluorosis and Kashin-Beck Disease.
PMID: 29317700
Sci Rep · 2018
0.60