Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000674581 | SCV000799941 | likely pathogenic | Alstrom syndrome | 2018-05-14 | criteria provided, single submitter | clinical testing | |
Invitae | RCV000674581 | SCV000937664 | pathogenic | Alstrom syndrome | 2021-10-08 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Experimental studies and prediction algorithms are not available or were not evaluated, and the functional significance of this variant is currently unknown. ClinVar contains an entry for this variant (Variation ID: 558330). This variant has not been reported in the literature in individuals affected with ALMS1-related conditions. This variant is present in population databases (rs758952057, ExAC 0.002%). This sequence change creates a premature translational stop signal (p.Ser269*) in the ALMS1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in ALMS1 are known to be pathogenic (PMID: 17594715). |