Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Foundation for Research in Genetics and Endocrinology, |
RCV003156409 | SCV003845181 | pathogenic | Mucopolysaccharidosis type 6 | 2023-03-18 | criteria provided, single submitter | clinical testing | A homozygous single base pair deletion in exon 2 of the ARSB gene that results in a frameshift and premature truncation of the protein 38 amino acids downstream to codon 146 (p.Trp146Cysfs*38) was detected. This variant has not been reported in the 1000 genomes and gnomAD databases. The in-silico prediction of the variant is damaging MutationTaster2. The reference region is conserved across species. In summary the variant meets our criteria to be classified as pathogenic. |