Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000681944 | SCV001794588 | likely pathogenic | not provided | 2020-08-27 | criteria provided, single submitter | clinical testing | In-frame deletion of 1 amino acids in a non-repeat region; Not observed at a significant frequency in large population cohorts (Lek et al., 2016); In silico analysis supports a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 11317350, 29277467, 15151504, 12955714, 11161832) |
Clinical Genomics, |
RCV002509508 | SCV002817338 | likely risk allele | Wolfram syndrome 1 | criteria provided, single submitter | research | Potent mutations in WFS1 gene are associated with Wolfram's syndrome, an autosomal recessive condition, which cause diabetes mellitus, diabetes insipidus, deafness and optic atrophy.However no sufficient evidence is found to ascertain the role of this particular variant rs1560418164 in Wolfram's syndrome yet. | |
Invitae | RCV000681944 | SCV004293158 | uncertain significance | not provided | 2023-12-31 | criteria provided, single submitter | clinical testing | This variant, c.1049_1051del, results in the deletion of 1 amino acid(s) of the WFS1 protein (p.Phe350del), but otherwise preserves the integrity of the reading frame. This variant is present in population databases (no rsID available, gnomAD 0.0009%). This variant has been observed in individual(s) with Wolfram syndrome (PMID: 11161832, 28432734). This variant is also known as c.1046delTCT (350delF). ClinVar contains an entry for this variant (Variation ID: 562461). Experimental studies and prediction algorithms are not available or were not evaluated, and the functional significance of this variant is currently unknown. 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. |
Gharavi Laboratory, |
RCV000681944 | SCV000809431 | likely pathogenic | not provided | 2018-09-16 | no assertion criteria provided | research |