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Monogenic hearing loss v5.49 RFC4 Ida Ertmanska Tag Q3_24_promote_green was removed from gene: RFC4.
Monogenic hearing loss v5.49 RFC4 Ida Ertmanska reviewed gene: RFC4: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance:
Monogenic hearing loss v5.48 RFC4 Ida Ertmanska Source Expert Review Green was added to RFC4.
Source NHS GMS was added to RFC4.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Monogenic hearing loss v4.57 RFC4 Achchuthan Shanmugasundram Classified gene: RFC4 as Amber List (moderate evidence)
Monogenic hearing loss v4.57 RFC4 Achchuthan Shanmugasundram Added comment: Comment on list classification: There is sufficient evidence available for the association of this gene with sensorineural hearing impairment. Hence, this gene should be promoted to green rating in the next GMS update.
Monogenic hearing loss v4.57 RFC4 Achchuthan Shanmugasundram Gene: rfc4 has been classified as Amber List (Moderate Evidence).
Monogenic hearing loss v4.56 RFC4 Achchuthan Shanmugasundram gene: RFC4 was added
gene: RFC4 was added to Monogenic hearing loss. Sources: Literature
Q3_24_promote_green tags were added to gene: RFC4.
Mode of inheritance for gene: RFC4 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: RFC4 were set to 39106866
Phenotypes for gene: RFC4 were set to sensorineural hearing loss disorder, MONDO:0020678
Review for gene: RFC4 was set to GREEN
Added comment: PMID:39106866 reported nine individuals (aged birth to 47 years) from eight unrelated families with a multisystem disorder.

They presented with muscle weakness/myopathy (9/9), motor incoordination/gait disturbance (8/8), delayed gross motor development (6/9), dysarthria (5/5), peripheral neuropathy (3/3 adults), bilateral sensorineural hearing impairment (6/9), decreased body weight (8/9), short stature (5/9), microcephaly (4/9), respiratory issues/insufficiency (6/9), cerebellar atrophy (4/9), pituitary hypoplasia (3/9).

They were identified with biallelic loss-of-function variants in RFC4 gene (3 frameshift, 2 splice site, 1 single AA duplication, 2 single AA deletions and 2 missense)

This gene has not yet been associated with relevant phenotypes either in OMIM or in Gene2Phenotype.
Sources: Literature