Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ce |
RCV000994556 | SCV001148168 | uncertain significance | not provided | 2019-04-01 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000994556 | SCV003469128 | pathogenic | not provided | 2022-06-28 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln895*) in the DNHD1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in DNHD1 are known to be pathogenic (PMID: 34932939). This variant is present in population databases (no rsID available, gnomAD 0.1%). This variant has not been reported in the literature in individuals affected with DNHD1-related conditions. ClinVar contains an entry for this variant (Variation ID: 806605). For these reasons, this variant has been classified as Pathogenic. |
Prevention |
RCV003392722 | SCV004111049 | uncertain significance | DNHD1-related disorder | 2023-06-06 | criteria provided, single submitter | clinical testing | The DNHD1 c.2683C>T variant is predicted to result in premature protein termination (p.Gln895*). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.12% of alleles in individuals of Latino descent in gnomAD (http://gnomad.broadinstitute.org/variant/11-6555088-C-T). Loss of function is not an established mechanism of DNHD1-related disease. Although we suspect that this variant may be pathogenic, at this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |