Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
SIB Swiss Institute of Bioinformatics | RCV000852309 | SCV000994942 | likely pathogenic | Capillary malformation-arteriovenous malformation 2 | 2019-03-29 | criteria provided, single submitter | curation | This variant is interpreted as a Likely pathogenic for Capillary malformation-arteriovenous malformation 2. The following ACMG Tag(s) were applied: PM2: Absent from controls (or at extremely low frequency if recessive) in Exome Sequencing Project, 1000 Genomes Project, or Exome Aggregation Consortium. PM1: Located in a mutational hot spot and/or critical and well-established functional domain (e.g.,active site of an enzyme) without benign variation. PP3: Multiple lines of computational evidence support a deleterious effect on the gene or gene product. PM6: Assumed de novo, but without confirmation of paternity and maternity. |
Center for Genomics, |
RCV002062235 | SCV002496109 | uncertain significance | Lymphatic malformation 7 | 2020-11-23 | criteria provided, single submitter | clinical testing | EPHB4 NM_004444.4 exon 15 p.Cys856Tyr (c.2567G>A): This variant has been reported in the literature in one individual with CM-AVM (Amyere 2017 PMID:28687708). This variant is not present in large control databases and is present in ClinVar (Variation ID:691534). Evolutionary conservation and computational predictive tools suggest that this variant may impact the protein. In summary, data on this variant is insufficient for disease classification. Therefore, the clinical significance of this variant is uncertain. |
Center for Genomics, |
RCV003224479 | SCV003919920 | uncertain significance | Lymphatic malformation 7; Capillary malformation-arteriovenous malformation 2 | 2021-03-30 | criteria provided, single submitter | clinical testing | EPHB4 NM_004444.4 exon 15 p.Cys856Tyr (c.2567G>A): This variant has been reported in the literature in one individual with CM-AVM (Amyere 2017 PMID:28687708). This variant is not present in large control databases and is present in ClinVar (Variation ID:691534). Evolutionary conservation and computational predictive tools suggest that this variant may impact the protein. In summary, data on this variant is insufficient for disease classification. Therefore, the clinical significance of this variant is uncertain. |