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Mendeliome v2.302 KCNH7 Zornitza Stark Classified gene: KCNH7 as Amber List (moderate evidence)
Mendeliome v2.302 KCNH7 Zornitza Stark Gene: kcnh7 has been classified as Amber List (Moderate Evidence).
Mendeliome v2.301 KCNH7 Zornitza Stark edited their review of gene: KCNH7: Changed rating: AMBER
Mendeliome v2.301 KCNH7 Zornitza Stark Marked gene: KCNH7 as ready
Mendeliome v2.301 KCNH7 Zornitza Stark Gene: kcnh7 has been classified as Green List (High Evidence).
Mendeliome v2.301 KCNH7 Zornitza Stark Classified gene: KCNH7 as Green List (high evidence)
Mendeliome v2.301 KCNH7 Zornitza Stark Gene: kcnh7 has been classified as Green List (High Evidence).
Mendeliome v2.300 KCNH7 Zornitza Stark gene: KCNH7 was added
gene: KCNH7 was added to Mendeliome. Sources: Literature
Mode of inheritance for gene: KCNH7 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: KCNH7 were set to 39634124
Phenotypes for gene: KCNH7 were set to Epilepsy, MONDO:0005027, KCNH7-related
Review for gene: KCNH7 was set to GREEN
Added comment: PMID 39634124 reports three individuals from three families with de novo heterozygous KCNH7 variants (c.83A>G p.K28R, c.1919A>G p.E640G, c.1324C>T p.R442X) presenting with early‑onset epilepsy (generalized tonic‑clonic seizures, focal motor seizures, West syndrome). Functional evidence is limited to a mouse ERG3 knockdown model and protein structural modelling. One of the variants, p.R442X, is present in 4 hets in gnomAD.
Sources: Literature