Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000667105 | SCV000791503 | uncertain significance | Mucopolysaccharidosis, MPS-III-B | 2017-05-17 | criteria provided, single submitter | clinical testing | |
Invitae | RCV001240804 | SCV001413779 | uncertain significance | Mucopolysaccharidosis, MPS-III-B; Charcot-Marie-Tooth disease, axonal type 2V | 2019-08-09 | criteria provided, single submitter | clinical testing | This variant, c.202_203insCCG, results in the insertion of 1 amino acid(s) to the NAGLU protein (p.Leu67_Gly68insAla), but otherwise preserves the integrity of the reading frame. The frequency data for this variant in the population databases is considered unreliable, as metrics indicate insufficient coverage at this position in the ExAC database. This variant has not been reported in the literature in individuals with NAGLU-related conditions. ClinVar contains an entry for this variant (Variation ID: 551933). In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |