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CAGSSS

Chr 1AR

isoleucyl-tRNA synthetase 2, mitochondrial

Also known as: CAGSSS, ILERS

The mitochondrial isoleucine-tRNA synthetase catalyzes the aminoacylation of tRNA by isoleucine within mitochondria, essential for mitochondrial protein synthesis. Autosomal recessive mutations cause a multisystem disorder characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia. This condition affects multiple organ systems reflecting the widespread cellular dependence on mitochondrial function.

OMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/CAGSSS?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CAGSSS · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

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Clinical Literature
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