Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001937990 | SCV002178628 | pathogenic | Growth hormone insensitivity with immune dysregulation 1, autosomal recessive | 2023-10-13 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Leu142Argfs*20) in the STAT5B gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in STAT5B are known to be pathogenic (PMID: 15827093). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with growth hormone insensitivity (PMID: 20538865). ClinVar contains an entry for this variant (Variation ID: 1408216). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. For these reasons, this variant has been classified as Pathogenic. |
Laboratorio de Genetica e Diagnostico Molecular, |
RCV001937990 | SCV003807362 | pathogenic | Growth hormone insensitivity with immune dysregulation 1, autosomal recessive | 2022-10-12 | criteria provided, single submitter | clinical testing | ACMG classification criteria: PVS1 very strong, PM2 moderated, PM3 moderated |