Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001291052 | SCV005184481 | likely pathogenic | Asphyxiating thoracic dystrophy 3 | 2024-05-31 | criteria provided, single submitter | clinical testing | Variant summary: DYNC2H1 c.625T>A (p.Phe209Ile) results in a non-conservative amino acid change located in the dynein heavy chain, tail domain (IPR013594) of the encoded protein sequence. Three of five in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 1.3e-05 in 223852 control chromosomes (gnomAD). c.625T>A has been reported in the literature in individuals affected with clinical features of short-rib thoracic dysplasia (Zhang_2018). These data indicate that the variant is likely to be associated with disease. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publications have been ascertained in the context of this evaluation (PMID: 19361615, 29068549). ClinVar contains an entry for this variant (Variation ID: 446586). Based on the evidence outlined above, the variant was classified as likely pathogenic. |
Dan Cohn Lab, |
RCV000516133 | SCV000611979 | pathogenic | Jeune thoracic dystrophy | 2017-06-01 | no assertion criteria provided | research | |
Dan Cohn Lab, |
RCV001291052 | SCV000611989 | pathogenic | Asphyxiating thoracic dystrophy 3 | 2017-06-01 | no assertion criteria provided | research | |
University of Washington Center for Mendelian Genomics, |
RCV001291052 | SCV001479410 | likely pathogenic | Asphyxiating thoracic dystrophy 3 | no assertion criteria provided | research | ||
University of Washington Center for Mendelian Genomics, |
RCV000516133 | SCV001479568 | likely pathogenic | Jeune thoracic dystrophy | no assertion criteria provided | research |