NOTCH2NLC

Chr 1AD

notch 2 N-terminal like C

Also known as: ETM6, NIID, OPDM3

Enables Notch binding activity. Involved in cerebral cortex development and positive regulation of Notch signaling pathway. Located in extracellular region. Implicated in essential tremor 6; neuronal intranuclear inclusion disease; and oculopharyngodistal myopathy 3. [provided by Alliance of Genome Resources, Jul 2025]

Primary Disease Associations & Inheritance

Neuronal intranuclear inclusion diseaseMIM #603472
AD
Oculopharyngodistal myopathy 3MIM #619473
AD
Tremor, hereditary essential, 6MIM #618866
AD
5
ClinVar variants
4
Pathogenic / LP
pLI score
3
Active trials
Clinical SummaryNOTCH2NLC
📋
ClinVar Variants
4 Pathogenic / Likely Pathogenic· 1 VUS of 5 total submissions
💊
Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

5 submitted variants in ClinVar

Classification Summary

Pathogenic4
VUS1
4
Pathogenic
1
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
4
Likely Pathogenic
0
VUS
1
Likely Benign
0
Benign
0
Total5

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

NOTCH2NLC · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

Neuronal intranuclear inclusion disease

MIM #603472

Molecular basis of disorder known

Autosomal dominant

Oculopharyngodistal myopathy 3

MIM #619473

Molecular basis of disorder known

Autosomal dominant

Tremor, hereditary essential, 6

MIM #618866

Molecular basis of disorder known

Autosomal dominant
Clinical Literature
Landmark / reviewRecent case evidence