Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000233494 | SCV000285836 | uncertain significance | Familial aplasia of the vermis; Meckel-Gruber syndrome | 2022-08-01 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with cysteine, which is neutral and slightly polar, at codon 1618 of the CC2D2A protein (p.Arg1618Cys). This variant is present in population databases (rs201219078, gnomAD 0.05%). This variant has not been reported in the literature in individuals affected with CC2D2A-related conditions. ClinVar contains an entry for this variant (Variation ID: 238282). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt CC2D2A protein function. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Fulgent Genetics, |
RCV000765763 | SCV000897151 | uncertain significance | COACH syndrome 1; Meckel syndrome, type 6; Joubert syndrome 9 | 2018-10-31 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV001329603 | SCV001521090 | uncertain significance | Joubert syndrome 9 | 2019-08-03 | criteria provided, single submitter | clinical testing | This variant was determined to be of uncertain significance according to ACMG Guidelines, 2015 [PMID:25741868]. |
Gene |
RCV001753692 | SCV002006031 | uncertain significance | not provided | 2019-07-23 | criteria provided, single submitter | clinical testing | In silico analysis, which includes protein predictors and evolutionary conservation, supports a deleterious effect; Has not been previously published as pathogenic or benign to our knowledge |
Fulgent Genetics, |
RCV005031812 | SCV005664627 | uncertain significance | Meckel syndrome, type 6; Joubert syndrome 9; COACH syndrome 2; Retinitis pigmentosa 93 | 2024-04-19 | criteria provided, single submitter | clinical testing |