Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001001050 | SCV001158171 | uncertain significance | not specified | 2019-02-09 | criteria provided, single submitter | clinical testing | The DMD c.9352G>A; p.Ala3118Thr variant (rs200928985), to our knowledge, is not reported in the medical literature but is reported as a variant of uncertain significance in ClinVar (Variation ID: 520520). This variant is found in the Genome Aggregation Database on 2 out of 183,500 alleles, including 1 hemizygote. The alanine at codon 3118 is moderately conserved and computational analyses (SIFT: tolerated, PolyPhen-2: probably damaging) predict conflicting effects of this variant on protein structure/function. Due to limited information, the clinical significance of the p.Ala3118Thr variant is uncertain at this time. |
Labcorp Genetics |
RCV001248680 | SCV001422185 | likely benign | Duchenne muscular dystrophy | 2024-12-15 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002372726 | SCV002684904 | uncertain significance | Cardiovascular phenotype | 2024-10-16 | criteria provided, single submitter | clinical testing | The p.A3118T variant (also known as c.9352G>A), located in coding exon 64 of the DMD gene, results from a G to A substitution at nucleotide position 9352. The alanine at codon 3118 is replaced by threonine, an amino acid with similar properties. This variant was described in a Duchenne/Becker muscular dystrophy cohort; however, clinical details were not provided (Kim MJ et al. J Mol Diagn, 2016 Mar;18:253-9). This amino acid position is highly conserved in available vertebrate species. In addition, the in silico prediction for this alteration is inconclusive. Based on data from gnomAD, the A allele has an overall frequency of 0.0011% (2/183500) total alleles studied, with 1 hemizygote(s) observed. The highest observed frequency was 0.0076% (1/13161) of African/African American alleles. Based on the available evidence, the clinical significance of this variant remains unclear. |
Natera, |
RCV001832317 | SCV002080171 | uncertain significance | Becker muscular dystrophy; Duchenne muscular dystrophy; Cardiomyopathy; Dystrophin deficiency | 2019-07-10 | no assertion criteria provided | clinical testing | |
Prevention |
RCV004738114 | SCV005354826 | uncertain significance | DMD-related disorder | 2024-07-04 | no assertion criteria provided | clinical testing | The DMD c.9352G>A variant is predicted to result in the amino acid substitution p.Ala3118Thr. This variant has been reported in a cohort of individuals suspected of Duchenne muscular dystrophy or Becker muscular dystrophy; however, detailed clinical information was not available (Kim et al. 2016. PubMed ID: 26743743). This variant is reported in 0.0076% of alleles in individuals of African descent in gnomAD, including one hemizygote in the database. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |