Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Illumina Laboratory Services, |
RCV001123321 | SCV001282145 | uncertain significance | Autosomal recessive congenital ichthyosis 2 | 2017-04-27 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). Publications were found based on this search. However, the evidence from the literature, in combination with allele frequency data from public databases where available, was not sufficient to rule this variant in or out of causing disease. Therefore, this variant is classified as a variant of unknown significance. |
Gene |
RCV005056911 | SCV005689973 | likely pathogenic | not provided | 2024-08-09 | criteria provided, single submitter | clinical testing | Observed in the homozygous state in two cousins from one family and in an unrelated individual with congenital ichthyosis from the published literature (PMID: 32253496, 33435499); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 32253496, 33435499, 34851365, 20222929) |
Institute for Human Genetics, |
RCV001123321 | SCV001477964 | pathogenic | Autosomal recessive congenital ichthyosis 2 | 2021-01-07 | no assertion criteria provided | clinical testing |