Activity

Filter

Cancel
Date Panel Item Activity
11 actions
Deafness and congenital structural abnormalities v1.34 KIAA0391 Achchuthan Shanmugasundram Classified gene: KIAA0391 as Green List (high evidence)
Deafness and congenital structural abnormalities v1.34 KIAA0391 Achchuthan Shanmugasundram Added comment: Comment on list classification: As there are at least four unrelated families reported with sensorineural hearing loss, this gene has been promoted to green rating on this panel.
Deafness and congenital structural abnormalities v1.34 KIAA0391 Achchuthan Shanmugasundram Gene: kiaa0391 has been classified as Green List (High Evidence).
Deafness and congenital structural abnormalities v1.33 KIAA0391 Achchuthan Shanmugasundram Tag new-gene-name tag was added to gene: KIAA0391.
Deafness and congenital structural abnormalities v1.33 KIAA0391 Achchuthan Shanmugasundram commented on gene: KIAA0391: The 'new-gene-name' tag has been added as the official gene symbol for KIAA0391 is PRORP. It is also known as MRPP3.
Deafness and congenital structural abnormalities v1.33 KIAA0391 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: OMIM phenotype accessed on 10 October 2025.
Deafness and congenital structural abnormalities v1.33 KIAA0391 Achchuthan Shanmugasundram Phenotypes for gene: KIAA0391 were changed from Combined oxidative phosphorylation deficiency 54, OMIM:619737 to Combined oxidative phosphorylation deficiency 54, OMIM:619737
Deafness and congenital structural abnormalities v1.32 KIAA0391 Achchuthan Shanmugasundram Phenotypes for gene: KIAA0391 were changed from Sensorineural hearing loss; primary ovarian insufficiency; leukodystrophy to Combined oxidative phosphorylation deficiency 54, OMIM:619737
Deafness and congenital structural abnormalities v1.31 KIAA0391 Achchuthan Shanmugasundram Publications for gene: KIAA0391 were set to PMID:34715011; 37558808
Deafness and congenital structural abnormalities v1.30 KIAA0391 Achchuthan Shanmugasundram reviewed gene: KIAA0391: Rating: GREEN; Mode of pathogenicity: None; Publications: 34715011, 37558808; Phenotypes: Combined oxidative phosphorylation deficiency 54, OMIM:619737; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Deafness and congenital structural abnormalities v1.27 KIAA0391 Bill Newman gene: KIAA0391 was added
gene: KIAA0391 was added to Deafness and congenital structural abnormalities. Sources: Literature
Mode of inheritance for gene: KIAA0391 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: KIAA0391 were set to PMID:34715011; 37558808
Phenotypes for gene: KIAA0391 were set to Sensorineural hearing loss; primary ovarian insufficiency; leukodystrophy
Penetrance for gene: KIAA0391 were set to Complete
Review for gene: KIAA0391 was set to GREEN
Added comment: Note this gene should be called PRORP (also known as MRPP3)

Biallelic hylomorphic missense variants in the metallonuclease domain associated with this phenotype
Sources: Literature