Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000214959 | SCV000271975 | uncertain significance | not specified | 2015-12-24 | criteria provided, single submitter | clinical testing | The c.560-9C>G variant in MITF has not been previously reported in individuals w ith hearing loss or Waardenburg syndrome, but has been identified in 6/64578 Eur opean chromosomes by the Exome Aggregation Consortium (ExAC, http://exac.broadin stitute.org). This frequency is not high enough to rule out a pathogenic role. The variant is located in the 3' splice region, and computational tools suggest an impact to splicing. However, this information is not predictive enough to det ermine pathogenicity. In summary, the clinical significance of the c.560-9C>G va riant is uncertain. |
Gene |
RCV001596994 | SCV001830863 | uncertain significance | not provided | 2024-06-14 | criteria provided, single submitter | clinical testing | In silico analysis supports a deleterious effect on splicing; Has not been previously published as pathogenic or benign to our knowledge |
Sema4, |
RCV002256126 | SCV002536418 | uncertain significance | Hereditary cancer-predisposing syndrome | 2021-07-13 | criteria provided, single submitter | curation | |
Fulgent Genetics, |
RCV002485390 | SCV002792620 | uncertain significance | Tietz syndrome; Waardenburg syndrome type 2A; Melanoma, cutaneous malignant, susceptibility to, 8; Coloboma, osteopetrosis, microphthalmia, macrocephaly, albinism, and deafness | 2021-09-08 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002519640 | SCV002961000 | uncertain significance | Tietz syndrome; Waardenburg syndrome type 2A; Melanoma, cutaneous malignant, susceptibility to, 8 | 2024-01-05 | criteria provided, single submitter | clinical testing | This sequence change falls in intron 5 of the MITF gene. It does not directly change the encoded amino acid sequence of the MITF protein. This variant is present in population databases (rs766938558, gnomAD 0.01%). This variant has not been reported in the literature in individuals affected with MITF-related conditions. ClinVar contains an entry for this variant (Variation ID: 228853). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. 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. |