ICD-10 Code E88.43 – Disorders of mitochondrial tRNA synthetases (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E88.43 – Disorders of mitochondrial tRNA synthetases
What it is
E88.43 identifies inherited disorders caused by abnormal mitochondrial tRNA synthetase function. These conditions affect mitochondrial protein production and can disrupt energy-dependent tissues such as muscle, brain, and other organs.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings in mitochondrial disease include muscle weakness, neurologic impairment, developmental delay, or multisystem involvement. You should code only when the record specifically links the condition to mitochondrial tRNA synthetase disorder.
When to use this code
Use E88.43 when the provider documents a disorder of mitochondrial tRNA synthetases, mitochondrial aminoacyl-tRNA synthetase deficiency, or a named related genetic diagnosis. It may appear in specialist notes, genetic testing summaries, or problem lists. If the chart only says “mitochondrial disorder,” check documentation for the specific subtype.
Do not use for
Do not use this code for nonspecific mitochondrial disease without mention of tRNA synthetase involvement. Do not assign it for acquired myopathy, general neuromuscular weakness, or symptoms alone.
Coding tip
Look for the exact genetic or molecular diagnosis in the assessment, not just a broad mitochondrial label.