Gene
TNNI3K
TNNI3 interacting kinase
- HGNC 19661(opens in a new tab)
- Ensembl gene ENSG00000116783(opens in a new tab)
- NCBI Gene 51086(opens in a new tab)
Summary
- Cytogenetic location
- 1p31.1
- Coordinates
- Chromosome 1: 74,235,387–74,544,428 (GRCh38)
- Exons
- 25 exons across 309 kb
- Protein
- 835 amino acids
- Inheritance
- Autosomal dominant
- Also known as
- CARK
- MANE Select transcript
- ENST00000326637.8NM_015978.3
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Progressive Cardiac Conduction Disease & Sick Sinus Syndrome
- Atrial Conduction DiseaseAutosomal dominant
A disorder in which electrical conduction through the upper chambers of the heart is disrupted, which can lead to atrial standstill and slow rhythms.
- 2 GenCC submitters rate it definitive or strong
- TNNI3K-Related Cardiac Conduction DiseaseAutosomal dominant
A condition caused by changes in TNNI3K that combines conduction disease and atrial rhythm disturbances, sometimes with dilated cardiomyopathy.
- 1 GenCC submitter rates it definitive or strong
What knowing changes
Knowing about it supports ECG and heart-muscle monitoring.
Dilated Cardiomyopathy
Enlargement and weakening of the left ventricle not explained by coronary artery or valve disease; inherited forms involve TTN, LMNA, RBM20, FLNC, BAG3, DSP, MYH7, TNNT2, DES, PLN and many other genes, and include Barth syndrome and the Emery-Dreifuss muscular dystrophies.
- ClinGen rates the link moderate
What knowing changes
Knowing a genetic cause supports earlier heart monitoring and family screening, and for genes with a high arrhythmia risk such as LMNA, FLNC and RBM20 it informs decisions about rhythm monitoring and implantable defibrillators.
Inheritance
- Autosomal dominant
- One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.
How TNNI3K is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Stretches short reads find hard
Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.
Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).
Nearby genes from Ensembl release 116, on GRCh38.
Exons make up 3 kb of the gene's 309 kb; 2,508 of those bases code for the protein.
Traits linked to TNNI3K
The Human Phenotype Ontology lists these as features of the rare conditions TNNI3K can cause. Each trait's page lists the other genes linked to it.
External links
Gene records
Transcript
Protein
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). TNNI3K gene: TNNI3 interacting kinase. Retrieved 17 October 2026, from https://dantelabs.com/genes/tnni3k
Dante Labs. TNNI3K gene: TNNI3 interacting kinase [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/tnni3k
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