Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV000015683 | SCV003841767 | pathogenic | Congenital reticular ichthyosiform erythroderma | 2023-02-23 | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v2.1.1 dataset. This variant was predicted to alter splicing and result in a loss or disruption of normal protein function. Multiple pathogenic loss-of-function variants are reported downstream of the variant. The variant has been reported to be associated with KRT10 related disorder (PMID: 20798280). Therefore, this variant is classified as Pathogenic according to the recommendation of ACMG/AMP guideline. |
OMIM | RCV000015683 | SCV000035948 | pathogenic | Congenital reticular ichthyosiform erythroderma | 2010-10-01 | no assertion criteria provided | literature only |