Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Rare Diseases, |
RCV005053124 | SCV005686753 | pathogenic | Autosomal recessive nonsyndromic hearing loss 77 | 2025-01-09 | criteria provided, single submitter | research | PVS1;PM3_Supporting;PM2_Supporting |
Labcorp Genetics |
RCV005105391 | SCV005742354 | pathogenic | not provided | 2024-02-19 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Asp1532Valfs*15) in the LOXHD1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in LOXHD1 are known to be pathogenic (PMID: 19732867, 21465660, 25792669). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with LOXHD1-related conditions. For these reasons, this variant has been classified as Pathogenic. |