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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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LNX2
ligand of numb-protein X 2
Chromosome 13 · 13q12.2
NCBI Gene: 222484Ensembl: ENSG00000139517.10HGNC: HGNC:20421UniProt: Q8N448
49PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingidentical protein bindingubiquitin-protein transferase activityPDZ domain bindingexostosisovarian neoplasmtrauma complicationAnxiety
✦AI Summary

LNX2 (ligand of numb-protein X 2) is a RING-type E3 ubiquitin ligase that regulates protein stability and localization through ubiquitin-mediated pathways 1. The protein contains multiple PDZ domains that mediate protein-protein interactions, distinguishing it from other E3 ligases 1. LNX2 functions as an E3 ubiquitin ligase by binding substrates through its PDZ domains and promoting their ubiquitination via its RING domain 2. Key substrates include the T-cell co-receptor CD8α, which LNX2 ubiquitinates leading to its endocytosis and lysosomal degradation 2. In the nervous system, LNX2 localizes at neuronal gap junctions formed by connexin36 and regulates synaptic transmission by promoting connexin36 ubiquitination and internalization 3. LNX2 also controls the presynaptic glycine transporter GlyT2, modulating glycinergic neurotransmission through PKC-mediated mechanisms 4. Disease relevance includes involvement in ALS/ALS-FTD pathology, where LNX2 expression patterns are altered in specific cortical neuron subtypes 5. Additionally, LNX2 serves as a host target for SARS-CoV-2 proteins, potentially contributing to viral pathogenesis 6. Clinically, LNX2's regulation of GlyT2 has implications for hyperekplexia and chr13 pain disorders 4.

Sources cited
1
LNX2 is a RING-type E3 ubiquitin ligase with multiple PDZ domains
PMID: 33333989
2
LNX2 ubiquitinates CD8α leading to its endocytosis and degradation
PMID: 22045731
3
LNX2 localizes at neuronal gap junctions and regulates connexin36 ubiquitination
PMID: 30295974
4
LNX2 regulates GlyT2 transporter and glycinergic neurotransmission
PMID: 31628376
5
LNX2 expression patterns are altered in ALS/ALS-FTD cortical neurons
PMID: 41803120
6
LNX2 is targeted by SARS-CoV-2 proteins
PMID: 33864728
Disease Associationsⓘ20
exostosisOpen Targets
0.19Weak
ovarian neoplasmOpen Targets
0.05Suggestive
trauma complicationOpen Targets
0.03Suggestive
AnxietyOpen Targets
0.02Suggestive
type 2 diabetes mellitusOpen Targets
0.01Suggestive
lung adenocarcinomaOpen Targets
0.01Suggestive
autosomal dominant cerebellar ataxiaOpen Targets
0.01Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
inflammatory bowel diseaseOpen Targets
0.01Suggestive
Q feverOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
mastitisOpen Targets
0.01Suggestive
diabetes mellitusOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
endocarditisOpen Targets
0.00Suggestive
spina bifidaOpen Targets
0.00Suggestive
hyperekplexiaOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HOXB2Shared pathway100%MOBPShared pathway100%SCRG1Shared pathway100%IRF2BPLShared pathway100%NRSN2Shared pathway100%GNG8Shared pathway100%
Tissue Expression6 tissues
Heart
100%
Liver
98%
Lung
75%
Brain
72%
Bone Marrow
34%
Ovary
24%
Gene Interaction Network
Click a node to explore
LNX2HOXB2MOBPSCRG1IRF2BPLNRSN2GNG8
PROTEIN STRUCTURE
Preparing viewer…
PDB5E1Y · 1.01 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.69LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.52 [0.39–0.69]
RankingsWhere LNX2 stands among ~20K protein-coding genes
  • #8,971of 20,598
    Most Researched49
  • #5,207of 17,882
    Most Constrained (LOEUF)0.69
Genes detectedLNX2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Electric-field-stimulated protein mechanics.
PMID: 27926732
Nature · 2016
1.00
2
Ligand of Numb proteins LNX1p80 and LNX2 interact with the human glycoprotein CD8α and promote its ubiquitylation and endocytosis.
PMID: 22045731
J Cell Sci · 2011
0.90
3
Identification and characterization of human PDZRN4L gene and mouse Pdzrn4l gene in silico.
PMID: 15138636
Int J Mol Med · 2004
0.80
4
Multi-modal dissection of cell-type specific TDP-43 pathology in the motor cortex.
PMID: 41803120
Nat Commun · 2026
0.70
5
E3 ubiquitin ligases LNX1 and LNX2 localize at neuronal gap junctions formed by connexin36 in rodent brain and molecularly interact with connexin36.
PMID: 30295974
Eur J Neurosci · 2018
0.60