Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001773078 | SCV002003794 | uncertain significance | not provided | 2021-04-02 | criteria provided, single submitter | clinical testing | In silico analysis supports that this missense variant does not alter protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Labcorp Genetics |
RCV001868634 | SCV002171483 | uncertain significance | Jeune thoracic dystrophy; Nephronophthisis | 2024-12-02 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 850 of the TTC21B protein (p.Ala850Val). This variant is present in population databases (rs199795649, gnomAD 0.02%). 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: 1314569). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is not expected to disrupt TTC21B protein function with a negative predictive value of 95%. 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, |
RCV002489807 | SCV002785571 | uncertain significance | Asphyxiating thoracic dystrophy 4; Nephronophthisis 12 | 2024-04-25 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004968272 | SCV005523477 | uncertain significance | Inborn genetic diseases | 2024-11-29 | criteria provided, single submitter | clinical testing | The c.2549C>T (p.A850V) alteration is located in exon 19 (coding exon 19) of the TTC21B gene. This alteration results from a C to T substitution at nucleotide position 2549, causing the alanine (A) at amino acid position 850 to be replaced by a valine (V). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Prevention |
RCV004734264 | SCV005363883 | uncertain significance | TTC21B-related disorder | 2024-09-25 | no assertion criteria provided | clinical testing | The TTC21B c.2549C>T variant is predicted to result in the amino acid substitution p.Ala850Val. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.020% of alleles in individuals of African descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |