Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Centre de Recherche et de Formation en Génétique Médicale et en Neurosciences, |
RCV004701244 | SCV005200387 | likely pathogenic | Lafora disease | 2024-08-30 | no assertion criteria provided | case-control | PP3 (Strong): MetaRNN = 0.978 is greater than 0.939 ⇒ strong pathogenic PM2 (Supporting): variant not found in gnomAD genomes, good gnomAD genomes coverage = 32.3. Variant not found in gnomAD exomes, good gnomAD exomes coverage = 83.6. PP1 (Supporting): Cosegregation with phenotype in multiple affected family members in gene NHLRC1 which is associated with Apraxia, Ataxia, Atonic Seizure, Atypical Absence Seizure, Bilateral Tonic-Clonic Seizure and 36 more, according to ClinGen Disease Validity, GenCC, Mondo and PanelApp. PP2 (Supporting): 28 out of 29 non-VUS missense variants in gene NHLRC1 are pathogenic = 96.6% which is more than threshold of 80.8%. |