Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
SIB Swiss Institute of Bioinformatics | RCV000536686 | SCV000883284 | likely pathogenic | Glycosylphosphatidylinositol biosynthesis defect 16 | 2018-10-15 | criteria provided, single submitter | curation | This variant is interpreted as Likely Pathogenic, for Glycosylphosphatidylinositol biosynthesis defect 16, autosomal recessive. The following ACMG Tag(s) were applied: PVS1-Moderate => PVS1 downgraded in strength to Moderate. PS3-Moderate => PS3 downgraded in strength to Moderate (https://www.ncbi.nlm.nih.gov/pubmed/27694521). PM2 => Recessive disease and allele present at low frequency in Exome Sequencing Project, 1000 Genomes Project, or Exome Aggregation Consortium. |
New York Genome Center | RCV000536686 | SCV001480231 | likely pathogenic | Glycosylphosphatidylinositol biosynthesis defect 16 | 2020-07-09 | criteria provided, single submitter | clinical testing | |
DASA | RCV000536686 | SCV002498800 | pathogenic | Glycosylphosphatidylinositol biosynthesis defect 16 | 2022-04-10 | criteria provided, single submitter | clinical testing | The c.61C>T;p.(Arg21)* variant creates a premature translational stop signal in the PIGC gene. It is expected to result in an absent or disrupted protein product -PVS1_moderate. Well-established in vitro or in vivo functional studies supportive of a damaging effect on the gene or gene product (PMID: 27694521) - PS3_supporting. This sequence change has been observed in affected individual(s) and ClinVar contains an entry for this variant (ClinVar ID: 471153; PMID: 27694521) - PS4. The variant is present at low allele frequencies population databases (rs115209243 – gnomAD 0.0007425%; ABraOM 0.000854 frequency - http://abraom.ib.usp.br) - PM2_supporting. The p.(Arg21*) was detected in trans with a pathogenic variant (PMID: 27694521) - PM3. The variant co-segregated with disease in multiple affected family members (PMID: 27694521) - PP1. In summary, the currently available evidence indicates that the variant is pathogenic |
OMIM | RCV000536686 | SCV000649374 | pathogenic | Glycosylphosphatidylinositol biosynthesis defect 16 | 2017-12-21 | no assertion criteria provided | literature only | |
Eurofins Ntd Llc |
RCV000729218 | SCV000856860 | uncertain significance | not provided | 2017-09-11 | flagged submission | clinical testing |