Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
SIB Swiss Institute of Bioinformatics | RCV000723367 | SCV001245382 | likely pathogenic | Intellectual disability, autosomal recessive 66 | 2020-02-14 | criteria provided, single submitter | curation | This variant is interpreted as likely pathogenic for Mental retardation, autosomal recessive 66. The following ACMG Tag(s) were applied: PM2, PVS1-Strong. |
Institute of Human Genetics, |
RCV000723367 | SCV001443032 | pathogenic | Intellectual disability, autosomal recessive 66 | 2020-03-01 | criteria provided, single submitter | clinical testing | Review of the variants reported in Reuter et al., 2017, PMID: 28097321: PVS1,PM2,PM3_Supporting |
3billion | RCV000723367 | SCV002058748 | pathogenic | Intellectual disability, autosomal recessive 66 | 2022-01-03 | criteria provided, single submitter | clinical testing | Stop-gained (nonsense): predicted to result in a loss or disruption of normal protein function through nonsense-mediated decay (NMD) or protein truncation. Multiple pathogenic variants are reported downstream of the variant (PVS1_VS).The variant has been reported at least twice as pathogenic/likely pathogenic with clinical assertions and evidence for the classification (ClinVar ID: VCV000592164, PMID:28097321). It is observed at an extremely low frequency in the gnomAD v2.1.1 dataset (total allele frequency: 0.000025, PM2_M). Therefore, this variant is classified as pathogenic according to the recommendation of ACMG/AMP guideline. |
Gene |
RCV003235375 | SCV003933250 | pathogenic | not provided | 2024-04-15 | criteria provided, single submitter | clinical testing | Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; This variant is associated with the following publications: (PMID: 28097321) |
OMIM | RCV000723367 | SCV000854767 | pathogenic | Intellectual disability, autosomal recessive 66 | 2021-12-22 | no assertion criteria provided | literature only |