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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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DNER
delta/notch like EGF repeat containing
Chromosome 2 · 2q36.3
NCBI Gene: 92737Ensembl: ENSG00000187957.9HGNC: HGNC:24456UniProt: Q8NFT8
41PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Receptor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
dendriteprotein bindingcentral nervous system developmentearly endosomeknee fracturetongue cancerbreast carcinomaAbnormality of the skeletal system
✦AI Summary

DNER (delta/notch like EGF repeat containing) functions as a target antigen for anti-Tr antibodies in paraneoplastic cerebellar ataxia. DNER was identified as the specific antigenic target of Tr antibodies, which are markers of cerebellar ataxia associated with Hodgkin's lymphoma 1. Anti-Tr/DNER antibody-associated cerebellar ataxia predominantly affects middle-aged males and presents as rapidly progressive isolated cerebellar dysfunction 2. The condition shows strong tumor association, with 91% of patients having an underlying malignancy, most commonly Hodgkin lymphoma 2. DNER antibodies are detected in both serum and cerebrospinal fluid of affected patients 3. While oncological treatment typically yields excellent responses (88% complete response), neurological prognosis remains poor, with only 41% of patients showing significant neurological improvement 2. The antibody testing for DNER shows high specificity and sensitivity, though positive predictive values can be moderate, particularly in serum testing 3. This autoimmune vestibulocerebellar syndrome requires early recognition and immunotherapy, as outcomes are better when treatment is initiated promptly 4. Current evidence is primarily derived from case reports and small series due to the rarity of this condition.

Sources cited
1
DNER was identified as the target antigen of Tr antibodies associated with cerebellar ataxia and Hodgkin's lymphoma
PMID: 23041955
2
Anti-Tr/DNER antibody-associated cerebellar ataxia predominantly affects middle-aged males with 91% tumor association and poor neurological prognosis
PMID: 34817790
3
DNER antibody testing shows high specificity and sensitivity with moderate positive predictive values
PMID: 39467237
4
Early immunotherapy improves outcomes in autoimmune vestibulocerebellar syndromes including anti-Tr/DNER conditions
PMID: 31958862
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
knee fractureOpen Targets
0.33Weak
tongue cancerOpen Targets
0.31Weak
breast carcinomaOpen Targets
0.30Weak
Abnormality of the skeletal systemOpen Targets
0.28Weak
cardiomyopathyOpen Targets
0.28Weak
respiratory tract infectious disorderOpen Targets
0.28Weak
estrogen-receptor positive breast cancerOpen Targets
0.27Weak
HER2 negative breast carcinomaOpen Targets
0.27Weak
progesterone-receptor positive breast cancerOpen Targets
0.27Weak
response to antineoplastic agentOpen Targets
0.27Weak
Abnormal pupillary functionOpen Targets
0.23Weak
tooth diseaseOpen Targets
0.22Weak
kidney diseaseOpen Targets
0.14Weak
HirsutismOpen Targets
0.13Weak
melanomaOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.10Weak
diffuse large B-cell lymphomaOpen Targets
0.10Suggestive
neoplasmOpen Targets
0.09Suggestive
acute myeloid leukemiaOpen Targets
0.09Suggestive
glioblastoma multiformeOpen Targets
0.09Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
BIN1Protein interaction87%DTX1Protein interaction87%RBPJProtein interaction81%PSEN1Protein interaction78%ARHGAP26Protein interaction74%AMPHProtein interaction72%
Tissue Expression6 tissues
Brain
100%
Heart
1%
Ovary
0%
Lung
0%
Liver
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
DNERBIN1DTX1RBPJPSEN1ARHGAP26AMPH
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8NFT8
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.56Moderately Constrained
pLIⓘ
0.21Tolerant
Observed/Expected LoF0.41 [0.31–0.56]
RankingsWhere DNER stands among ~20K protein-coding genes
  • #9,996of 20,598
    Most Researched41
  • #3,650of 17,882
    Most Constrained (LOEUF)0.56 · top quartile
Genes detectedDNER
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
PMID: 37582614
Neurol Neuroimmunol Neuroinflamm · 2023
1.00
2
Autoimmune brainstem encephalitis: Clinical associations, outcomes, and proposed diagnostic criteria.
PMID: 39708293
Ann Clin Transl Neurol · 2025
0.90
3
Long-Term Cognitive, Functional, and Patient-Reported Outcomes in Patients With Anti-NMDAR Encephalitis.
PMID: 39566012
Neurology · 2024
0.80
4
Transcriptomics-Based Liquid Biopsy for Early Detection of Recurrence in Locally Advanced Gastric Cancer.
PMID: 39556695
Adv Sci (Weinh) · 2024
0.70
5
Autoimmune Vestibulocerebellar Syndromes.
PMID: 31958862
Semin Neurol · 2020
0.60