Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
SIB Swiss Institute of Bioinformatics | RCV001256178 | SCV001432974 | pathogenic | CEBALID syndrome | 2020-07-29 | criteria provided, single submitter | curation | This variant is interpreted as Pathogenic for CEBALID syndrome, autosomal dominant. The following ACMG Tag(s) were applied: Absent from controls (or at extremely low frequency if recessive) in Exome Sequencing Project, 1000 Genomes Project, or Exome Aggregation Consortium (PM2); Located in a mutational hot spot and/or critical and well-established functional domain (e.g., active site of an enzyme) without benign variation (PM1); Protein length changes as a result of in-frame deletions/insertions in a nonrepeat region or stop-loss variants (PM4); De novo (paternity and maternity confirmed) (PS2). |
Gene |
RCV001773573 | SCV001994506 | uncertain significance | not provided | 2019-08-27 | criteria provided, single submitter | clinical testing | Nonsense variant predicted to result in protein truncation, although loss-of-function variants have not been reported downstream of this position in the protein; Not observed in large population cohorts (Lek et al., 2016); Variants in candidate genes are classified as variants of uncertain significance in accordance with ACMG guidelines (Richards et al., 2015); Has not been previously published as pathogenic or benign to our knowledge; This variant is associated with the following publications: (PMID: 31834374) |
Laboratoire de Genetique Biologique, |
RCV001256178 | SCV004101262 | pathogenic | CEBALID syndrome | 2023-11-08 | criteria provided, single submitter | clinical testing | The same variant was previously described by Mak et al., 2020 (PMID: 31834374). The patient analyzed by our Institution presents the same phenotype. |
University of Washington Center for Mendelian Genomics, |
RCV001258020 | SCV001434834 | likely pathogenic | MN1 C-terminal truncation (MCTT) syndrome | no assertion criteria provided | research |