Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000574758 | SCV000673608 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-08-16 | criteria provided, single submitter | clinical testing | The p.D135N variant (also known as c.403G>A), located in coding exon 5 of the MAX gene, results from a G to A substitution at nucleotide position 403. The aspartic acid at codon 135 is replaced by asparagine, an amino acid with highly similar properties. This amino acid position is well conserved in available vertebrate species. In addition, the in silico prediction for this alteration is inconclusive. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Labcorp Genetics |
RCV001042399 | SCV001206077 | uncertain significance | Hereditary pheochromocytoma-paraganglioma | 2024-01-11 | criteria provided, single submitter | clinical testing | This sequence change replaces aspartic acid, which is acidic and polar, with asparagine, which is neutral and polar, at codon 135 of the MAX protein (p.Asp135Asn). This variant is present in population databases (rs201312694, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with MAX-related conditions. ClinVar contains an entry for this variant (Variation ID: 485704). Advanced modeling performed at Invitae incorporating data from internal and/or published experimental studies (Invitae) indicates that this missense variant is not expected to disrupt MAX 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. |
Baylor Genetics | RCV004569274 | SCV005060753 | uncertain significance | Pheochromocytoma | 2024-02-25 | criteria provided, single submitter | clinical testing |