Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000457169 | SCV000551597 | benign | Perlman syndrome | 2023-12-19 | criteria provided, single submitter | clinical testing | |
Genetic Services Laboratory, |
RCV001821286 | SCV002071996 | uncertain significance | not specified | 2021-11-19 | criteria provided, single submitter | clinical testing | DNA sequence analysis of the DIS3L2 gene demonstrated a sequence change, c.1722G>T, in exon 14 that results in an amino acid change, p.Glu574Asp. This sequence change has been described in the gnomAD database with a frequency of 0.038% in the non-Finnish European subpopulation (dbSNP rs191608083). The p.Glu574Asp change affects a poorly conserved amino acid residue located in a domain of the DIS3L2 protein that is not known to be functional. In-silico pathogenicity prediction tools (SIFT, PolyPhen2, Align GVGD, REVEL) provide contradictory results for the p.Glu574Asp substitution. This sequence change does not appear to have been previously described in individuals with DIS3L2-related disorders. Due to insufficient evidences and the lack of functional studies, the clinical significance of the p.Glu574Asp change remains unknown at this time. |
Sema4, |
RCV000457169 | SCV002532402 | uncertain significance | Perlman syndrome | 2021-08-13 | criteria provided, single submitter | curation | |
Ambry Genetics | RCV002523345 | SCV003688052 | uncertain significance | Inborn genetic diseases | 2021-11-19 | criteria provided, single submitter | clinical testing | The c.1722G>T (p.E574D) alteration is located in exon 14 (coding exon 13) of the DIS3L2 gene. This alteration results from a G to T substitution at nucleotide position 1722, causing the glutamic acid (E) at amino acid position 574 to be replaced by an aspartic acid (D). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Mayo Clinic Laboratories, |
RCV003480643 | SCV004226021 | uncertain significance | not provided | 2022-07-21 | criteria provided, single submitter | clinical testing | BP4 |