Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000056601 | SCV000321833 | pathogenic | not provided | 2024-05-23 | criteria provided, single submitter | clinical testing | Located in the highly conserved head domain of KRT5, which is intolerant to change; variants in this region interfere with proper keratin intermediate filament assembly and function, resulting in skin fragility and/or hyperkeratosis (PMID: 21176769); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 16786515, 23450297, 36809127) |
Institute of Medical Genetics and Applied Genomics, |
RCV000056601 | SCV001446718 | pathogenic | not provided | 2020-10-23 | criteria provided, single submitter | clinical testing | |
Revvity Omics, |
RCV000056601 | SCV002016401 | pathogenic | not provided | 2021-07-20 | criteria provided, single submitter | clinical testing | |
Epithelial Biology; Institute of Medical Biology, |
RCV000056601 | SCV000087714 | not provided | not provided | no assertion provided | not provided |