Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Division of Biology and Genetics, |
RCV001290341 | SCV001478260 | pathogenic | Autosomal recessive cutis laxa type 2B | criteria provided, single submitter | clinical testing | ||
Kasturba Medical College, |
RCV004762051 | SCV005341002 | pathogenic | PYCR1- related autosomal recessive cutis laxa | criteria provided, single submitter | clinical testing | This canonical splice-site variant would result in aberrant splicing leading to either a truncated protein product or the mRNA of the transcript would be degraded through nonsense-mediated decay. Previously this variant, c.67+2T>A in a compound heterozygous state with another missense change in PYCR1 has been reported in a patient with PYCR1-related autosomal recessive cutis laxa (Ritelli et al, 2017). |