Total submissions: 14
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000758141 | SCV000886633 | likely benign | Usher syndrome | 2018-10-22 | reviewed by expert panel | curation | The filtering allele frequency of the c.905G>A (p.Arg302His) variant in the MYO7A gene is 0.4% for European chromosomes by gnomAD (587/126276 with 95% CI), which is a high enough frequency to be classified as likely benign based on the thresholds defined by the ClinGen Hearing Loss Expert Panel for autosomal recessive hearing loss variants (BS1). While this variant has been reported in several individuals with Usher syndrome, in 2 individuals it was identified in cis with another pathogenic MYO7A variant (BP2; PMID 8900236). This variant has also been reported in at least 2 additional individuals with Usher syndrome who had alternate genetic etiologies identified (PMID: 25468891). In addition, in vitro functional evidence from one study suggests that the Arg302His variant had little effect on motor activity of MYO7A (BS3_Supporting PMID: 18700726). In summary, this variant meets criteria to be classified as likely benign. ACMG/AMP criteria applied, as specified by the Hearing Loss Expert Panel : BS1, BP2, BS3_Supporting. |
Laboratory for Molecular Medicine, |
RCV000036251 | SCV000059903 | benign | not specified | 2010-05-06 | criteria provided, single submitter | clinical testing | This variant has been identified in 4.2% of controls(rs41298135) and functional studies do not show an impact to protein function (Watanabe 2008). |
Eurofins Ntd Llc |
RCV000036251 | SCV000232931 | likely benign | not specified | 2014-12-16 | criteria provided, single submitter | clinical testing | |
Illumina Laboratory Services, |
RCV000386045 | SCV000374225 | likely benign | Usher syndrome type 1 | 2017-04-27 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). Publications were found based on this search. The evidence from the literature, in combination with allele frequency data from public databases where available, was sufficient to determine this variant is unlikely to cause disease. Therefore, this variant is classified as likely benign. |
Illumina Laboratory Services, |
RCV000282374 | SCV000374226 | likely benign | Autosomal dominant nonsyndromic hearing loss 11 | 2017-04-27 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases allowed determination this variant is unlikely to cause disease. Therefore, this variant is classified as likely benign. |
Illumina Laboratory Services, |
RCV000337254 | SCV000374227 | likely benign | Autosomal recessive nonsyndromic hearing loss 2 | 2017-04-27 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases allowed determination this variant is unlikely to cause disease. Therefore, this variant is classified as likely benign. |
Gene |
RCV000835045 | SCV000976822 | likely benign | not provided | 2018-05-30 | criteria provided, single submitter | clinical testing | This variant is considered likely benign or benign based on one or more of the following criteria: it is a conservative change, it occurs at a poorly conserved position in the protein, it is predicted to be benign by multiple in silico algorithms, and/or has population frequency not consistent with disease. |
Labcorp Genetics |
RCV000835045 | SCV001030679 | benign | not provided | 2025-02-03 | criteria provided, single submitter | clinical testing | |
Ce |
RCV000835045 | SCV001371320 | likely benign | not provided | 2025-02-01 | criteria provided, single submitter | clinical testing | MYO7A: BS2 |
Fulgent Genetics, |
RCV002490355 | SCV002801697 | likely benign | Autosomal dominant nonsyndromic hearing loss 11; Autosomal recessive nonsyndromic hearing loss 2; Usher syndrome type 1 | 2021-11-19 | criteria provided, single submitter | clinical testing | |
Institute of Human Genetics, |
RCV004814885 | SCV005073369 | likely benign | Retinal dystrophy | 2008-01-01 | criteria provided, single submitter | clinical testing | |
Breakthrough Genomics, |
RCV000835045 | SCV005217875 | likely benign | not provided | criteria provided, single submitter | not provided | ||
OMIM | RCV000012626 | SCV000032861 | pathogenic | Usher syndrome type 1B | 1996-11-01 | no assertion criteria provided | literature only | |
Natera, |
RCV000012626 | SCV001459062 | benign | Usher syndrome type 1B | 2020-04-16 | no assertion criteria provided | clinical testing |