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Genotype–phenotype correlation in IARS2‐related diseases: A case report and review of literature

Jariya Upadia, Yuwen Li, Nicolette Walano, Stephen Deputy, Kelly K. Gajewski, Hans C. Andersson

2022Clinical Case Reports12 citationsDOIOpen Access PDF

Abstract

Abstract Isoleucyl‐tRNA synthetase 2 (IARS2) encodes mitochondrial isoleucine‐tRNA synthetase. Pathogenic variants in the IARS2 gene are associated with mitochondrial disease. We report a female with IARS2 compound heterozygous variants, p.Val499Glyfs*14 and p.Arg784Trp who presented with infantile spasms, Leigh disease and Wolff‐Parkinson White (WPW) pattern. This report expands the phenotypic spectrum of IARS2 ‐related disease.

Topics & Concepts

PhenotypeGeneticsMedicineGenotypeDiseaseMitochondrial diseaseGeneCompound heterozygosityClinical phenotypeTransfer RNABiologyMitochondrial DNAPathologyRNAMitochondrial Function and PathologyMetabolism and Genetic DisordersATP Synthase and ATPases Research
Genotype–phenotype correlation in IARS2‐related diseases: A case report and review of literature | Litcius