Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000699403 | SCV000828111 | likely benign | Muscle AMP deaminase deficiency | 2023-09-27 | criteria provided, single submitter | clinical testing | |
Neuberg Centre For Genomic Medicine, |
RCV000699403 | SCV005400821 | uncertain significance | Muscle AMP deaminase deficiency | criteria provided, single submitter | clinical testing | The observed missense c.323C>T(p.Thr108Ile) variant in AMPD1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Thr108Ile variant is present with allele frequency 0.02% in gnomAD Exomes. This variant has been submitted to the ClinVar database as Likely Benign. Multiple lines of computational evidence (SIFT - Tolerated and MutationTaster - Polymorphism) predict no damaging effect on protein structure and function for this variant. The reference amino acid of p.Thr108Ile in AMPD1 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Thr at position 108 is changed to a Ile changing protein sequence and it might alter its composition and physico-chemical properties. Additional functional stuides will be required to prove the pathogenicity of this variant. For these reasons, this variant has been classified as Variant of Uncertain Significance (VUS). |