Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV003323341 | SCV004028531 | likely pathogenic | Pyruvate kinase deficiency of red cells | 2023-08-16 | criteria provided, single submitter | clinical testing | The c.829G>T variant is not present in not present in publicly available population databases like 1000 Genomes, ExAC, EVS, gnomAD, Indian Exome Database or our in-house exome database. This variant has neither been published in literature for PKLR-related conditions nor reported to the clinical databases like ClinVar, Human Genome Mutation Database (HGMD) or OMIM, in any affected individuals. In-silico pathogenicity prediction programs like MutationTaster2, CADD, Varsome, Franklin, InterVar etc predicted the c.829G>T variant to be likely deleterious. This variant creates a premature stop signal at the 277th amino acid position of wild-type transcript that may either result in translation of a truncated protein or cause nonsense mediated decay of the mRNA. This individuals another heterozygous variant (c.657_677del) in PKLR gene. |