Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV003466338 | SCV004209961 | likely pathogenic | Charlevoix-Saguenay spastic ataxia | 2023-06-23 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003588923 | SCV004295464 | likely benign | Spastic paraplegia | 2024-01-04 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004783067 | SCV005394564 | uncertain significance | not specified | 2024-09-12 | criteria provided, single submitter | clinical testing | Variant summary: SACS c.1894C>T (p.Arg632Trp) results in a non-conservative amino acid change in the encoded protein sequence. Three of four in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 8e-06 in 250800 control chromosomes. c.1894C>T has been reported in the literature in individuals affected with Autosomal Recessive Spastic Ataxia Of Charlevoix-Saguenay (examples: Gazulle_2011 and Longo_2021). These data indicate that the variant may be associated with disease. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publications have been ascertained in the context of this evaluation (PMID: 21665375, 34649874). ClinVar contains an entry for this variant (Variation ID: 2678528). Based on the evidence outlined above, the variant was classified as VUS-possibly pathogenic. |