Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001361384 | SCV001557359 | pathogenic | not provided | 2023-10-15 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Ile131Leufs*6) in the RINT1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in RINT1 are known to be pathogenic (PMID: 31204009). This variant is present in population databases (rs771302691, gnomAD 0.006%). This variant has not been reported in the literature in individuals affected with RINT1-related conditions. ClinVar contains an entry for this variant (Variation ID: 1053086). For these reasons, this variant has been classified as Pathogenic. |
Ambry Genetics | RCV004036802 | SCV002621001 | uncertain significance | not specified | 2023-06-18 | criteria provided, single submitter | clinical testing | The c.390delC variant, located in coding exon 4 of the RINT1 gene, results from a deletion of one nucleotide at nucleotide position 390, causing a translational frameshift with a predicted alternate stop codon (p.I131Lfs*6). This alteration is expected to result in premature protein truncation or nonsense-mediated mRNA decay. However, the gene-disease association for RINT1 is limited. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |