Cystic kidney disease

Gene: CFAP47

Green List (high evidence)

CFAP47 (cilia and flagella associated protein 47)
EnsemblGeneIds (GRCh38): ENSG00000165164
EnsemblGeneIds (GRCh37): ENSG00000165164
CFAP47 is in 2 panels

2 reviews

Achchuthan Shanmugasundram (Genomics England Curator)

Green List (high evidence)

The rating of this gene has been updated to green following NHS Genomic Medicine Service approval.
Created: 11 Mar 2026, 2:25 p.m. | Last Modified: 11 Mar 2026, 2:25 p.m.
Panel Version: 8.7
Comment on list classification: There is sufficient evidence available (three unrelated cases and functional work) for the promotion of this gene to green rating in the next GMS update.
Created: 23 Dec 2024, 7:21 p.m. | Last Modified: 23 Dec 2024, 7:21 p.m.
Panel Version: 7.10
PMID:39698362 reported the identification of three different rare missense variants in CFAP47 gene in three male patients with polycystic kidney disease. Some functional work is also available in support of the association.

This gene has not yet been associated with polycystic kidney disease phenotype either in OMIM or in Gene2Phenotype.
Created: 23 Dec 2024, 7:20 p.m. | Last Modified: 23 Dec 2024, 7:20 p.m.
Panel Version: 7.7

Mode of inheritance
X-LINKED: hemizygous mutation in males, biallelic mutations in females

Phenotypes
polycystic kidney disease, MONDO:0020642

Publications

Dmitrijs Rots (Children's Clinical University Hospital)

Green List (high evidence)

Three cases & some functional work described in PMID: 38633811 (now published).
Sources: Literature
Created: 3 Dec 2024, 11:44 a.m.

Mode of inheritance
X-LINKED: hemizygous mutation in males, biallelic mutations in females

Phenotypes
PKD

Publications

Mode of pathogenicity
Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments

Details

Mode of Inheritance
X-LINKED: hemizygous mutation in males, biallelic mutations in females
Sources
  • Expert Review Green
  • NHS GMS
Phenotypes
  • polycystic kidney disease, MONDO:0020642
Tags
gene-checked
Clinvar variants
Variants in CFAP47
Penetrance
unknown
Publications
Mode of Pathogenicity
Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments
Panels with this gene

History Filter Activity

19 Mar 2026, Gel status: 3

Added Tag

Ida Ertmanska (Genomics England Curator)

Tag gene-checked tag was added to gene: CFAP47.

11 Mar 2026, Gel status: 3

Removed Tag

Arina Puzriakova (Genomics England Curator)

Tag Q4_24_promote_green was removed from gene: CFAP47.

11 Mar 2026, Gel status: 3

Added New Source, Added New Source, Status Update

Arina Puzriakova (Genomics England Curator)

Source NHS GMS was added to CFAP47. Source Expert Review Green was added to CFAP47. Rating Changed from Amber List (moderate evidence) to Green List (high evidence)

23 Dec 2024, Gel status: 2

Added Tag

Achchuthan Shanmugasundram (Genomics England Curator)

Tag Q4_24_promote_green tag was added to gene: CFAP47.

23 Dec 2024, Gel status: 2

Entity classified by Genomics England curator

Achchuthan Shanmugasundram (Genomics England Curator)

Gene: cfap47 has been classified as Amber List (Moderate Evidence).

23 Dec 2024, Gel status: 0

Set Phenotypes

Achchuthan Shanmugasundram (Genomics England Curator)

Phenotypes for gene: CFAP47 were changed from PKD to polycystic kidney disease, MONDO:0020642

23 Dec 2024, Gel status: 0

Set publications

Achchuthan Shanmugasundram (Genomics England Curator)

Publications for gene: CFAP47 were set to PMID: 38633811

3 Dec 2024, Gel status: 0

Created, Added New Source, Set mode of inheritance, Set publications, Set Phenotypes, Set penetrance, Set mode of pathogenicity

Dmitrijs Rots (Children's Clinical University Hospital)

gene: CFAP47 was added gene: CFAP47 was added to Cystic kidney disease. Sources: Literature Mode of inheritance for gene: CFAP47 was set to X-LINKED: hemizygous mutation in males, biallelic mutations in females Publications for gene: CFAP47 were set to PMID: 38633811 Phenotypes for gene: CFAP47 were set to PKD Penetrance for gene: CFAP47 were set to unknown Mode of pathogenicity for gene: CFAP47 was set to Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments Review for gene: CFAP47 was set to GREEN