NKX2-6

Chr 8

NK2 homeobox 6

Also known as: CSX2, CTHM, NKX2F, NKX4-2

This gene encodes a homeobox-containing protein that belongs to the NK-2 homeobox family. This protein is a vertebrate homolog of Drosophila homeobox-containing protein called 'tinman', which has been shown to be essential for development of the heart-like dorsal vessel. In conjunction with related gene, NKX2-5, this gene may play a role in both pharyngeal and cardiac embryonic development. Mutations in this gene are associated with persistent truncus arteriosus.[provided by RefSeq, Aug 2011]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Conotruncal heart malformationsMIM #217095
Persistent truncus arteriosusMIM #217095

Clinical highlights

Gene-disease validity (ClinGen)
congenital heart disease · ARLimitednot for standalone diagnostic reporting
0
Active trials
4
Pubs (1 yr)
P/LP submissions
P/LP missense
1.03
LOEUF
DN
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.03LOEUF
pLI 0.019
Z-score 1.51
OE 0.45 (0.221.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.26Z-score
OE missense 0.94 (0.831.07)
165 obs / 174.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.45 (0.221.03)
00.351.4
Missense OE?0.94 (0.831.07)
00.61.4
Synonymous OE?0.76
01.21.6
LoF obs/exp: 4 / 8.8Missense obs/exp: 165 / 174.8Syn Z: 1.72

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NKX2-6 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →