Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV003156227 | SCV003845809 | likely pathogenic | not provided | 2022-09-23 | criteria provided, single submitter | clinical testing | Identified as heterozygous in a child with multiple episodes of ketotic hypoglycemia, ketoacidosis, and ketonuria in published literature (van Hasselt et al., 2014); Frameshift variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 25390740) |
OMIM | RCV000145412 | SCV000192501 | pathogenic | Monocarboxylate transporter 1 deficiency, autosomal dominant | 2014-11-13 | no assertion criteria provided | literature only |