Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Research Unit of Respiratory Disease, |
RCV001375490 | SCV001439387 | uncertain significance | Male infertility; Recurrent sinusitis; Situs inversus; Bronchiectasis | 2020-10-19 | no assertion criteria provided | research | Whole exome and Sanger sequencing identified a homozygous missense variant (NM_018963.5:1016T>C, p.(Leu339Ser) in the BRWD1 gene in a consanguineous family. The variant was absent in the 1000 Genomes Project (1000G), the NHLBI "Grand Opportunity" Exome Sequencing Project (GO-ESP). The two variants were predicted to be deleterious by Mutation taster, SIFT, PolyPhen-2, and CADD (PHRED-scaled score = 28.5). According to the American College of Medical Genetics and Genomics (ACMG) guideline interpretation, this missense variant in BRWD1 was evaluated as uncertain significance. This is the first report of BRWD1 gene links to the MMAF and likely PCD phenotype. |