Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001058984 | SCV001223587 | pathogenic | Primary ciliary dyskinesia 28 | 2019-12-22 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in SPAG1 are known to be pathogenic (PMID: 24055112). This variant has been observed in individual(s) with primary ciliary dyskinesia (PMID: 24055112). ClinVar contains an entry for this variant (Variation ID: 88684). This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Asp848Ilefs*10) in the SPAG1 gene. It is expected to result in an absent or disrupted protein product. |
OMIM | RCV000074367 | SCV000105974 | pathogenic | Autosomal dominant nocturnal frontal lobe epilepsy 5 | 2013-10-03 | no assertion criteria provided | literature only |