Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV003464679 | SCV004205795 | pathogenic | Autosomal recessive osteopetrosis 1 | 2023-03-02 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003779127 | SCV004639328 | pathogenic | not provided | 2023-02-22 | criteria provided, single submitter | clinical testing | Studies have shown that disruption of this splice site results in abnormally spliced transcripts and introduces a premature termination codon (PMID: 10888887). The resulting mRNA is expected to undergo nonsense-mediated decay. This sequence change affects an acceptor splice site in intron 14 of the TCIRG1 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 individuals with autosomal recessive osteopetrosis (PMID: 10888887, 15300850; Invitae). For these reasons, this variant has been classified as Pathogenic. |