long QT syndrome 10
MONDO:0012737Mondo
Findings
No curated finding names long QT syndrome 10 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Any long QT syndrome in which the cause of the disease is a mutation in the SCN4B gene.
Definition from the Mondo Disease Ontology (MONDO:0012737), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Adult onset · Juvenile onset · Childhood onset
HPO, annotations 2026-09-02
Features
4 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Sudden cardiac deathHPOHP:0001645
- 2 of 10 reported patients
- Atrioventricular blockHPOHP:0001678
- Prolonged QT intervalHPOHP:0001657
- T-wave alternansHPOHP:0012266
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- SCN4BHGNC:10592
- Limited · Labcorp Genetics (formerly Invitae) · Unknown · 2020
- Limited · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2020
- Limited · Laboratory for Molecular Medicine · Unknown · 2020
Where it sits
- A kind of
Other names
4 names
Resolves to: long QT syndrome 10
- Also called
- long QT syndrome caused by mutation in SCN4Blong QT syndrome type 10LQT10SCN4B long QT syndrome