Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000377802 | SCV000332750 | uncertain significance | not provided | 2015-07-28 | criteria provided, single submitter | clinical testing | |
ARUP Laboratories, |
RCV000377802 | SCV000885339 | uncertain significance | not provided | 2018-03-25 | criteria provided, single submitter | clinical testing | The DYNC2H1 c.6093T>G; p.Asp2031Glu variant (rs372641908, ClinVar variant ID 281777), to our knowledge, is not reported in the medical literature, gene specific variation databases, nor has it been previously identified by our laboratory. This variant is listed in the genome Aggregation Database (gnomAD) with an African population frequency of 0.1% (identified on 34 out of 23,996 chromosomes). The aspartic acid at position 2031 is highly conserved, considering 28 species, and computational analyses of the effects of the p.Asp2031Glu variant on protein structure and function predict a deleterious effect (SIFT: damaging, PolyPhen-2: probably damaging). Based on the available information, the clinical significance of the p.Asp2031Glu variant cannot be determined with certainty. |
Labcorp Genetics |
RCV001459433 | SCV001663272 | likely benign | Jeune thoracic dystrophy | 2024-11-27 | criteria provided, single submitter | clinical testing | |
Genetic Services Laboratory, |
RCV001820808 | SCV002070177 | uncertain significance | not specified | 2020-03-17 | criteria provided, single submitter | clinical testing | The sequence change, c.6093T>G, in exon 38 results in an amino acid change, p.Asp2031Glu. This sequence change does not appear to have been previously described in patients with DYNC2H1-related disorders and has been described in the gnomAD database with a low population frequency of 0.14% in the African subpopulation (dbSNP rs372641908). The p.Asp2031Glu change affects a highly conserved amino acid residue located in a domain of the DYNC2H1 protein that is known to be functional. The p.Asp2031Glu substitution appears to be deleterious using several in-silico pathogenicity prediction tools (SIFT, PolyPhen2, Align GVGD, REVEL).Due to these contrasting evidences and the lack of functional studies, the clinical significance of the p.Asp2031Glu change remains unknown at this time. |
Gene |
RCV000377802 | SCV002512947 | uncertain significance | not provided | 2022-04-26 | criteria provided, single submitter | clinical testing | In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Ambry Genetics | RCV002519099 | SCV003702478 | uncertain significance | Inborn genetic diseases | 2024-08-05 | criteria provided, single submitter | clinical testing | The c.6093T>G (p.D2031E) alteration is located in exon 38 (coding exon 38) of the DYNC2H1 gene. This alteration results from a T to G substitution at nucleotide position 6093, causing the aspartic acid (D) at amino acid position 2031 to be replaced by a glutamic acid (E). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |