Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
SIB Swiss Institute of Bioinformatics | RCV000779605 | SCV001146828 | likely pathogenic | Neurodevelopmental disorder with or without variable brain abnormalities; NEDBA | 2019-10-02 | criteria provided, single submitter | curation | This variant is interpreted as a Likely pathogenic for Neurodevelopmental disorder with or without variable brain abnormalities, autosomal dominant. The following ACMG Tag(s) were applied: PM2, PM6, PP3, PS3-Moderate, PS4-Supporting. |
Institute of Human Genetics, |
RCV000779605 | SCV001335371 | likely pathogenic | Neurodevelopmental disorder with or without variable brain abnormalities; NEDBA | 2019-02-07 | criteria provided, single submitter | clinical testing | This variant was identified as de novo (maternity and paternity confirmed). |
Institute of Human Genetics Munich, |
RCV000779605 | SCV001429927 | pathogenic | Neurodevelopmental disorder with or without variable brain abnormalities; NEDBA | 2020-03-23 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV001266867 | SCV001445047 | pathogenic | Inborn genetic diseases | 2019-07-03 | criteria provided, single submitter | clinical testing | |
Victorian Clinical Genetics Services, |
RCV000779605 | SCV002766744 | pathogenic | Neurodevelopmental disorder with or without variable brain abnormalities; NEDBA | 2022-03-31 | criteria provided, single submitter | clinical testing | Based on the classification scheme VCGS_Germline_v1.3.4, this variant is classified as Pathogenic. Following criteria are met: 0102 - Loss of function is a known mechanism of disease in this gene and is associated with neurodevelopmental disorder with or without variable brain abnormalities (MIM#618443). (I) 0107 - This gene is associated with autosomal dominant disease. (I) 0200 - Variant is predicted to result in a missense amino acid change from arginine to cysteine. (I) 0251 - This variant is heterozygous. (I) 0301 - Variant is absent from gnomAD (both v2 and v3). (SP) 0501 - Missense variant consistently predicted to be damaging by multiple in silico tools or highly conserved with a major amino acid change. (SP) 0600 - Variant is located in the annotated WD40 repeated domain (DECIPHER). (I) 0801 - This variant has strong previous evidence of pathogenicity in unrelated individuals. It has been reported de novo in at least five individuals with neurodevelopmental disorder with or without variable brain abnormalities (MIM#618443) and consistently classified as pathogenic by diagnostic laboratories in ClinVar (DECIPHER, PMID: 30612693, 30945334). (SP) Legend: (SP) - Supporting pathogenic, (I) - Information, (SB) - Supporting benign |
OMIM | RCV000779605 | SCV000916283 | pathogenic | Neurodevelopmental disorder with or without variable brain abnormalities; NEDBA | 2019-05-23 | no assertion criteria provided | literature only |