Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003388927 | SCV005061088 | uncertain significance | Acroerythrokeratoderma | criteria provided, single submitter | clinical testing | The observed missense c.211C>T(p.Arg71Cys) variant in SLURP1 gene has been reported previously in homozygous state in individual(s) affected with mal de Meleda (MDM) disease (Radiono S, et al., 2017; Karaca Z, et al., 2021). The p.Arg71Cys variant has been reported with allele frequency of 0.0004% in gnomAD Exomes. This variant has not been submitted to the ClinVar database. The amino acid Arg at position 71 is changed to a Cys changing protein sequence and it might alter its composition and physico-chemical properties. Multiple lines of computational evidence predict a damaging effect on protein structure and function for this variant. For these reasons, this variant has been classified as a Variant of Uncertain Significance (VUS). | |
Dermatology, |
RCV003388927 | SCV003921121 | pathogenic | Acroerythrokeratoderma | no assertion criteria provided | clinical testing |