Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Fulgent Genetics, |
RCV000008244 | SCV002776931 | pathogenic | Tumoral calcinosis, hyperphosphatemic, familial, 1 | 2021-09-01 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003546453 | SCV004270632 | pathogenic | not provided | 2023-11-05 | criteria provided, single submitter | clinical testing | This sequence change affects a donor splice site in intron 9 of the GALNT3 gene. RNA analysis indicates that disruption of this splice site induces altered splicing and may result in an absent or disrupted protein product. This variant is not present in population databases (gnomAD no frequency). Disruption of this splice site has been observed in individual(s) with GALNT3-related conditions (PMID: 17311862). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 7801). Studies have shown that disruption of this splice site results in activation of a cryptic splice site and introduces a premature termination codon (PMID: 17311862). The resulting mRNA is expected to undergo nonsense-mediated decay. For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000008244 | SCV000028451 | pathogenic | Tumoral calcinosis, hyperphosphatemic, familial, 1 | 2007-05-01 | no assertion criteria provided | literature only |