Total submissions: 8
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001584520 | SCV001813018 | pathogenic | not provided | 2021-12-09 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate a damaging effect with reduced DGAT1 protein levels observed in cells transfected with DGAT1 gene with this variant (van Rijn et al., 2018); In-frame deletion of 1 amino acids in a non-repeat region; In silico analysis, which includes protein predictors and evolutionary conservation, supports a deleterious effect; This variant is associated with the following publications: (PMID: 31778854, 33144682, 33607125, 29604290) |
Revvity Omics, |
RCV000660629 | SCV002024066 | likely pathogenic | Congenital diarrhea 7 with exudative enteropathy | 2021-09-07 | criteria provided, single submitter | clinical testing | |
Genetics and Molecular Pathology, |
RCV000660629 | SCV002761505 | likely pathogenic | Congenital diarrhea 7 with exudative enteropathy | 2020-07-30 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001584520 | SCV003460779 | pathogenic | not provided | 2024-04-25 | criteria provided, single submitter | clinical testing | This variant, c.629_631del, results in the deletion of 1 amino acid(s) of the DGAT1 protein (p.Ser210del), but otherwise preserves the integrity of the reading frame. This variant is present in population databases (rs782577883, gnomAD 0.01%). This variant has been observed in individual(s) with protein-losing enteropathy (PMID: 29604290, 31778854, 33607125). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 548013). Studies have shown that this variant alters DGAT1 gene expression (PMID: 29604290). For these reasons, this variant has been classified as Pathogenic. |
Fulgent Genetics, |
RCV000660629 | SCV005681660 | pathogenic | Congenital diarrhea 7 with exudative enteropathy | 2024-06-08 | criteria provided, single submitter | clinical testing | |
Mayo Clinic Laboratories, |
RCV000660629 | SCV000782751 | uncertain significance | Congenital diarrhea 7 with exudative enteropathy | 2017-11-15 | flagged submission | clinical testing | |
Laboratorio de Genetica e Diagnostico Molecular, |
RCV002252199 | SCV002523741 | uncertain significance | See cases | 2020-06-24 | flagged submission | clinical testing | ACMG classification criteria: PM2, PM4, PP3 |
OMIM | RCV000660629 | SCV002757898 | pathogenic | Congenital diarrhea 7 with exudative enteropathy | 2022-11-30 | no assertion criteria provided | literature only |