Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clinical Genetics, |
RCV003330373 | SCV004037473 | pathogenic | Cystinuria | 2023-06-22 | no assertion criteria provided | clinical testing | The SLC3A1 variant c.1431_1434dupAACT, p.(Tyr479Asnfs*19), has not yet been described, but it is known that "loss-of-function" is a known pathomechanism in the SLC3A1 gene, e.g., c.1754_1755delAG, p.(Glu585Alafs*24) (Gaildrat et al. 2017 and HGMD Professional 2023.1, among others). The variant is not listed in control databases (dbSNP, gnomAD v2.1.1). It is a reading frame mutation that leads to premature translational arrest and thus either loss of function of the affected allele due to premature degradation of the mutant mRNA or to a truncated protein with reduced or aberrant function. We classify the SLC3A1 variant c.1431_1434dupAACT, p.(Tyr479Asnfs*19), as pathogenic. |