Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genetic Services Laboratory, |
RCV001820602 | SCV002067994 | uncertain significance | not specified | 2020-04-30 | criteria provided, single submitter | clinical testing | DNA sequence analysis of the SDCCAG8 gene demonstrated a sequence change, c.415T>C, in exon 4 that results in an amino acid change, p.Cys139Arg. This sequence change has been described in the gnomAD database with a low population frequency of 0.001770% (dbSNP rs769333887). The p.Cys139Arg change affects a highly conserved amino acid residue located in a domain of the SDCCAG8 protein that is not known to be functional. In-silico pathogenicity prediction tools (SIFT, PolyPhen2, Align GVGD, REVEL) provide contradictory results for the p.Cys139Arg substitution. This sequence change does not appear to have been previously described in patients with SDCCAG8-related disorders. Due to these contrasting evidences and the lack of sufficient evidences, the clinical significance of the p.Cys139Arg change remains unknown at this time. |
Fulgent Genetics, |
RCV002506854 | SCV002815744 | uncertain significance | Senior-Loken syndrome 7; Bardet-Biedl syndrome 16 | 2022-02-17 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002545155 | SCV003705436 | uncertain significance | Inborn genetic diseases | 2022-12-16 | criteria provided, single submitter | clinical testing | The c.415T>C (p.C139R) alteration is located in exon 4 (coding exon 4) of the SDCCAG8 gene. This alteration results from a T to C substitution at nucleotide position 415, causing the cysteine (C) at amino acid position 139 to be replaced by an arginine (R). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |