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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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LSG1
large 60S subunit nuclear export GTPase 1
Chromosome 3 · 3q29
NCBI Gene: 55341Ensembl: ENSG00000041802.12HGNC: HGNC:25652UniProt: Q9H089
105PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneTransporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
membraneGTP bindingnuclear bodynuclear exportneurodegenerative diseasejoint diseasecellulitisabscess
✦AI Summary

LSG1 is a conserved GTPase essential for 60S ribosomal subunit maturation and cytoplasmic export 1. Its primary function involves mediating NMD3 release from the pre-60S subunit after nuclear export, facilitating proper ribosome assembly 2. Beyond ribosome biogenesis, LSG1 localizes to the endoplasmic reticulum where it tethers 60S subunits and regulates translation of select proteins 3. LSG1 interacts with VAP proteins through a noncanonical FFAT-like motif, enabling its ER localization independent of its GTPase function 4. LSG1 inhibition triggers cellular senescence not through ribosomal depletion but via endoplasmic reticulum homeostasis disruption and cholesterol biosynthesis pathway upregulation 2. The GTPase domain and acidic domain are functionally critical, as demonstrated in Drosophila studies showing LSG1 ortholog NS3 regulates neuroblast proliferation and cell polarity 5. LSG1's evolutionary expansion correlates with eukaryotic compartmentalization, with members localized to ER, nucleus, and other organelles 1. Dysregulation of LSG1 or related lncRNA (Lnc-LSG1) may contribute to renal cell carcinoma metastasis 6, suggesting LSG1 inhibition could have therapeutic potential in cancer management.

Sources cited
1
LSG1 is an essential GTPase predominantly located in the endoplasmic reticulum and Cajal bodies
PMID: 16209721
2
LSG1 GTPase inhibition causes senescence through ER disruption and cholesterol biosynthesis upregulation, not ribosome depletion
PMID: 31148378
3
LSG1 tethers 60S ribosomal subunits to the ER and regulates translation of select proteins
PMID: 40048539
4
LSG1 binds VAPs via noncanonical FFAT-like motif and is required for NMD3 recycling from pre-60S subunits
PMID: 39133101
5
NS3 (LSG1 ortholog) requires GTP-binding and acidic domains for neuroblast proliferation and proper cell polarity
PMID: 29679561
6
Lnc-LSG1 may stimulate renal cell carcinoma metastasis
PMID: 36614082
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.50Moderate
joint diseaseOpen Targets
0.22Weak
cellulitisOpen Targets
0.07Suggestive
abscessOpen Targets
0.06Suggestive
placenta praeviaOpen Targets
0.06Suggestive
urethral syndromeOpen Targets
0.06Suggestive
autonomic nervous system diseaseOpen Targets
0.06Suggestive
Abnormality of the integumentOpen Targets
0.05Suggestive
peripheral neuropathyOpen Targets
0.05Suggestive
connective tissue diseaseOpen Targets
0.05Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.04Suggestive
hyperpituitarismOpen Targets
0.04Suggestive
complex regional pain syndromeOpen Targets
0.02Suggestive
ovarian neoplasmOpen Targets
0.02Suggestive
alcohol drinkingOpen Targets
0.02Suggestive
bacteriemiaOpen Targets
0.01Suggestive
clear cell renal carcinomaOpen Targets
0.01Suggestive
infectionOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
heart failureOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RPL8Protein interaction100%RPL23AProtein interaction100%RPL37AProtein interaction100%RPL24Protein interaction100%RPL30Protein interaction100%RPL4Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
64%
Brain
49%
Lung
47%
Ovary
47%
Heart
43%
Gene Interaction Network
Click a node to explore
LSG1RPL8RPL23ARPL37ARPL24RPL30RPL4
PROTEIN STRUCTURE
Preparing viewer…
PDB6LSR · 3.13 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.84LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.67 [0.53–0.84]
RankingsWhere LSG1 stands among ~20K protein-coding genes
  • #4,539of 20,598
    Most Researched105 · top quartile
  • #7,265of 17,882
    Most Constrained (LOEUF)0.84
Genes detectedLSG1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A subcellular map of translational machinery composition and regulation at the single-molecule level.
PMID: 40048539
Science · 2025
1.00
2
The Ribosome Assembly Factor LSG1 Interacts with Vesicle-Associated Membrane Protein-Associated Proteins (VAPs).
PMID: 39133101
Mol Cell Biol · 2024
0.90
3
Inhibitory effect of probiotic Bacillus spp. isolated from the digestive tract of Rhynchocypris Lagowskii on the adhesion of common pathogenic bacteria in the intestinal model.
PMID: 35691482
Microb Pathog · 2022
0.80
4
The Role of Long Noncoding RNA (lncRNAs) Biomarkers in Renal Cell Carcinoma.
PMID: 36614082
Int J Mol Sci · 2022
0.70
5
Inhibition of the 60S ribosome biogenesis GTPase LSG1 causes endoplasmic reticular disruption and cellular senescence.
PMID: 31148378
Aging Cell · 2019
0.60