Primary ciliary disorders

Gene: DNAH9

Green List (high evidence)

DNAH9 (dynein axonemal heavy chain 9)
EnsemblGeneIds (GRCh38): ENSG00000007174
EnsemblGeneIds (GRCh37): ENSG00000007174
OMIM: 603330, Gene2Phenotype
DNAH9 is in 6 panels

2 reviews

Achchuthan Shanmugasundram (Genomics England Curator)

Green List (high evidence)

Comment on list classification: There is sufficient evidence available (eight unrelated cases and functional studies) for the promotion of this gene to green rating on this panel.
Created: 10 Oct 2025, 1:50 p.m. | Last Modified: 10 Oct 2025, 1:50 p.m.
Panel Version: 1.55
There are a total of eight unrelated families reported with primary ciliary dyskinesia (PCD) and biallelic DNAH9 variants. There is also functional evidence available in support of the association.

This gene has been associated with 'Ciliary dyskinesia, primary, 40' phenotype in OMIM (MIM #618300, OMIM record accessed on 10 October 2025). This gene is also classified with a 'Definitive' rating for ciliary dyskinesia, primary, 40 (MONDO:0032664) phenotype by the Motile Ciliopathy expert panel in ClinGen (https://search.clinicalgenome.org/CCID:008406)
Created: 10 Oct 2025, 1:49 p.m. | Last Modified: 10 Oct 2025, 1:49 p.m.
Panel Version: 1.51

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Phenotypes
Ciliary dyskinesia, primary, 40, OMIM:618300; ciliary dyskinesia, primary, 40, MONDO:0032664

Publications

Simon Thomas (Wessex Regional Genetics Laboratory)

Green List (high evidence)

Discussed on Respiratory TC.
Sources: Literature
Created: 20 Feb 2019, 10:15 a.m.

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Publications

Details

Mode of Inheritance
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
Phenotypes
  • Ciliary dyskinesia, primary, 40, OMIM:618300
  • ciliary dyskinesia, primary, 40, MONDO:0032664
OMIM
603330
Clinvar variants
Variants in DNAH9
Penetrance
None
Publications
Panels with this gene

History Filter Activity

10 Oct 2025, Gel status: 3

Entity classified by Genomics England curator

Achchuthan Shanmugasundram (Genomics England Curator)

Gene: dnah9 has been classified as Green List (High Evidence).

10 Oct 2025, Gel status: 3

Entity classified by Genomics England curator

Achchuthan Shanmugasundram (Genomics England Curator)

Gene: dnah9 has been classified as Green List (High Evidence).

10 Oct 2025, Gel status: 0

Set Phenotypes

Achchuthan Shanmugasundram (Genomics England Curator)

Phenotypes for gene: DNAH9 were changed from to Ciliary dyskinesia, primary, 40, OMIM:618300; ciliary dyskinesia, primary, 40, MONDO:0032664

10 Oct 2025, Gel status: 0

Set publications

Achchuthan Shanmugasundram (Genomics England Curator)

Publications for gene: DNAH9 were set to PMID: 30471717; 30471718

20 Feb 2019, Gel status: 0

Created, Added New Source, Set mode of inheritance, Set publications

Simon Thomas (Wessex Regional Genetics Laboratory)

gene: DNAH9 was added gene: DNAH9 was added to Primary ciliary disorders. Sources: Literature Mode of inheritance for gene: DNAH9 was set to BIALLELIC, autosomal or pseudoautosomal Publications for gene: DNAH9 were set to PMID: 30471717; 30471718 Review for gene: DNAH9 was set to GREEN