Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV003334459 | SCV004042823 | uncertain significance | Laron-type isolated somatotropin defect | 2023-10-06 | criteria provided, single submitter | clinical testing | The c.309T>A variant is not present in publicly available population databases like 1000 Genomes, EVS, ExAC, gnomAD, Indian Exome Database or our in-house exome database. This variant has neither been published in literature in individuals affected with GHR-related conditions nor reported to the clinical databases like ClinVar, Human Genome Mutation Database (HGMD) or OMIM. In-silico pathogenicity programs like Polyphen-2, MutationTaster2, CADD etc predicted this variant to be likely deleterious, however these predictions were not confirmed by published functional studies. This variant is located in a mutational hotspot region of the gene and a different amino acid change in the same codon (Asp103Asn) has been previously observed in a child affected with Laron syndrome [PMID: 29025428]. |