Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Illumina Laboratory Services, |
RCV000779557 | SCV000916231 | uncertain significance | Primary immunodeficiency with natural-killer cell deficiency and adrenal insufficiency | 2017-04-28 | criteria provided, single submitter | clinical testing | The MCM4 gene is the only gene associated with natural killer cell and glucocorticoid deficiency with DNA repair defect. The MCM4 c.685_686insA (p.Tyr229Ter) variant is a frameshift variant and is predicted to cause a truncation of the protein. Frequency information is not available from the 1000 Genomes Project, the Exome Sequencing Project, nor the Exome Aggregation Consortium. Based on the disease prevalence, disease penetrance, and inheritance mode, this variant could not be ruled out of causing disease. A literature search was performed for the gene, cDNA change, and amino acid change. No publications were found based on this search. Due to the potential impact of frameshift variants and the lack of clarifying evidence, this variant is classified as a variant of unknown significance, but suspicious for pathogenicity. |