Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001008519 | SCV001168291 | likely pathogenic | not provided | 2019-01-09 | criteria provided, single submitter | clinical testing | A variant that is likely pathogenic has been identified in the LAMB1 gene. The c.313_314delCC variant has not been published as a pathogenic variant, nor has it been reported as a benign variant to our knowledge. The c.313_314delCC variant in the LAMB1 gene causes a frameshift starting with codon Proline 105, changes this amino acid to a Lysine residue and creates a premature Stop codon at position 4 of the new reading frame, denoted p.Pro105LysfsX4. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.313_314delCC variant is not observed at a significant frequency in large population cohorts (Lek et al., 2016). Therefore, this variant is likely pathogenic; however, the possibility that it is benign cannot be excluded. |