Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000483626 | SCV000571319 | uncertain significance | not provided | 2025-01-13 | criteria provided, single submitter | clinical testing | In-frame deletion of 5 amino acids in a non-repeat region; In silico analysis supports a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Labcorp Genetics |
RCV001851222 | SCV002175366 | uncertain significance | Jeune thoracic dystrophy; Nephronophthisis | 2024-01-22 | criteria provided, single submitter | clinical testing | This variant, c.2777_2791del, results in the deletion of 5 amino acid(s) of the TTC21B protein (p.Arg926_Ala930del), but otherwise preserves the integrity of the reading frame. This variant is present in population databases (rs757389950, gnomAD 0.1%). This variant has not been reported in the literature in individuals affected with TTC21B-related conditions. ClinVar contains an entry for this variant (Variation ID: 421970). Experimental studies and prediction algorithms are not available or were not evaluated, and the functional significance of this variant is currently unknown. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Fulgent Genetics, |
RCV005018811 | SCV005651235 | uncertain significance | Asphyxiating thoracic dystrophy 4; Nephronophthisis 12 | 2024-03-13 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004735564 | SCV005347677 | uncertain significance | TTC21B-related disorder | 2024-08-16 | no assertion criteria provided | clinical testing | The TTC21B c.2777_2791del15 variant is predicted to result in an in-frame deletion (p.Arg926_Ala930del). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.12% of alleles in individuals of African descent in gnomAD. Although we suspect that this variant may be benign, the clinical significance of this variant is classified as uncertain at this time due to insufficient functional and genetic evidence. |