Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000803517 | SCV000943395 | pathogenic | Aculeiform cataract | 2021-08-15 | criteria provided, single submitter | clinical testing | This variant disrupts the p.Arg15 amino acid residue in CRYGD. Other variant(s) that disrupt this residue have been observed in individuals with CRYGD-related conditions (PMID: 19668596, 28450710), suggesting that it is a clinically significant residue. As a result, variants that disrupt this residue are likely to be causative of disease. This sequence change replaces arginine with cysteine at codon 15 of the CRYGD protein (p.Arg15Cys). The arginine residue is highly conserved and there is a large physicochemical difference between arginine and cysteine. This variant is not present in population databases (ExAC no frequency). This variant has been observed to segregate with congenital and juvenile onset cataract in families (PMID: 16446699, 9927684 ). This variant is also known as c.34C>T, p.R14C in the literature. ClinVar contains an entry for this variant (Variation ID: 16937). This variant has been reported to affect the CRYGD protein function (PMID: 19382745, 10688888 ). For these reasons, this variant has been classified as Pathogenic. |
Gene |
RCV003328551 | SCV004035767 | pathogenic | not provided | 2023-03-17 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate a damaging effect (Pande et al., 2000); Not observed at significant frequency in large population cohorts (gnomAD); This variant has been reported in the published literature using alternate nomenclature R14C; This variant is associated with the following publications: (PMID: 19382745, 19007775, 20577655, 29652984, 18035564, 26732753, 32899552, 21423869, 24384146, 29222017, 10688888, 23954869, 34150533, 19668596, 24465161, 20508808, 16446699, 32148946, 9927684) |
OMIM | RCV000018445 | SCV000038727 | pathogenic | Cataract 4 multiple types | 2007-09-01 | no assertion criteria provided | literature only |