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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CNIH2
cornichon family AMPA receptor auxiliary protein 2
Chromosome 11 · 11q13.2
NCBI Gene: 254263Ensembl: ENSG00000174871.11HGNC: HGNC:28744UniProt: Q6PI25
20PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingregulation of AMPA receptor activitypostsynaptic membranesynaptic transmission, glutamatergicneurodegenerative diseasemathematical abilitygoutinsomnia
✦AI Summary

CNIH2 is an auxiliary protein that regulates AMPA receptor (AMPAR) trafficking and gating properties. It promotes AMPAR targeting to the cell membrane and synapses while modulating their activation, deactivation, and desensitization rates 1. Structurally, CNIH2 destabilizes the desensitized state of AMPAR complexes and stabilizes polyamine binding to the ion channel selectivity filter, while attenuating polyamine block and reducing antiepileptic drug potency 2. CNIH2 enhances AMPAR tetramerization through transmembrane domain interactions, facilitating receptor biogenesis and surface expression more effectively than other auxiliary subunits 3. Beyond synaptic function, CNIH2 serves as a COPII cargo receptor in the secretory pathway, promoting trafficking of proteins through ER-Golgi compartments 4. Pathologically, elevated CNIH2 expression is associated with poor prognosis in esophageal squamous cell carcinoma and prostate cancer. In esophageal cancer, CPSF3-mediated alternative polyadenylation increases CNIH2 expression by eliminating miR-125a-5p binding sites, promoting tumor progression 5. Conversely, knockdown of CNIH2 in prostate cancer cells inhibits proliferation, migration, and invasion, with low CNIH2 expression correlating with improved progression-free survival 6. These findings establish CNIH2 as both a critical synaptic regulator and potential oncogenic driver in specific cancer contexts.

Sources cited
1
CNIH2-3 are auxiliary proteins that control AMPAR trafficking, conductance and gating of channels
PMID: 26255765
2
CNIH2 destabilizes the desensitized state of AMPAR complexes, stabilizes polyamine binding, and attenuates polyamine block and antiepileptic drug perampanel potency
PMID: 37653241
3
CNIH2 enhances AMPAR tetramerization through transmembrane domain interactions and promotes surface expression more effectively than other auxiliary subunits
PMID: 37673338
4
Cornichon proteins including CNIH2 function as COPII cargo receptors promoting protein trafficking through the secretory pathway
PMID: 38836370
5
CPSF3-induced ESCC progression is mediated by CNIH2 upregulation through alternative polyadenylation that eliminates miR-125a-5p binding sites
PMID: 38718887
6
Low CNIH2 expression associates with better progression-free survival in prostate cancer; CNIH2 knockdown inhibits cancer cell proliferation, migration, invasion and tumor growth
PMID: 39433941
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.38Weak
mathematical abilityOpen Targets
0.11Weak
goutOpen Targets
0.10Weak
insomniaOpen Targets
0.10Suggestive
prostate cancerOpen Targets
0.05Suggestive
attention deficit hyperactivity disorderOpen Targets
0.04Suggestive
hereditary attention deficit-hyperactivity disorderOpen Targets
0.04Suggestive
epilepsy, progressive myoclonic, 12Open Targets
0.04Suggestive
Potocki-Lupski syndromeOpen Targets
0.03Suggestive
schizophrenia 15Open Targets
0.03Suggestive
Phelan-McDermid syndromeOpen Targets
0.03Suggestive
Familial isolated hypoparathyroidismOpen Targets
0.03Suggestive
hypoparathyroidism, familial isolated 1Open Targets
0.03Suggestive
glycine encephalopathy 1Open Targets
0.03Suggestive
smoking initiationOpen Targets
0.02Suggestive
ataxia telangiectasiaOpen Targets
0.02Suggestive
uterine leiomyosarcomaOpen Targets
0.02Suggestive
esophageal squamous cell carcinomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
triple-negative breast cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GRIK2Protein interaction99%SYNDIG1Protein interaction99%DLG4Protein interaction94%NT5C1AProtein interaction88%GRIA1Protein interaction84%GRIA2Protein interaction84%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
76%
Ovary
2%
Liver
2%
Lung
2%
Heart
1%
Gene Interaction Network
Click a node to explore
CNIH2GRIK2SYNDIG1DLG4NT5C1AGRIA1GRIA2
PROTEIN STRUCTURE
Preparing viewer…
PDB8SS7 · 2.76 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.65LoF Tolerant
pLIⓘ
0.69Intermediate
Observed/Expected LoF0.35 [0.20–0.65]
RankingsWhere CNIH2 stands among ~20K protein-coding genes
  • #14,133of 20,598
    Most Researched20
  • #4,719of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedCNIH2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
CPSF3 regulates alternative polyadenylation of CNIH2 to promote esophageal squamous cell carcinoma progression.
PMID: 38718887
Cancer Lett · 2024
1.00
2
Expression and role of CNIH2 in prostate cancer.
PMID: 39433941
Sci Rep · 2024
0.90
3
Advances in the pharmacology of lGICs auxiliary subunits.
PMID: 26255765
Pharmacol Res · 2015
0.80
4
Modulation of GluA2-γ5 synaptic complex desensitization, polyamine block and antiepileptic perampanel inhibition by auxiliary subunit cornichon-2.
PMID: 37653241
Nat Struct Mol Biol · 2023
0.70
5
The Role of Cornichons in the Biogenesis and Functioning of Monovalent-Cation Transport Systems.
PMID: 38836370
Physiol Res · 2024
0.60