mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes

ORPHA: 3091362 Treatments Available

Overview

human disease

Available Treatments (2)

DrugFormStatusCountriesLead Time
Sodium Phenylbutyrate
Orphan
Tablets, PowderFDA Approved4
triheptanoin
Orphan
oral liquid, 1000mg/mLFDA Approved, EMA Approved621d

Classification & Codes

Orphanet Code

ORPHA:309136
mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes
OrphanetORPHA:309136
Treatments2 drug(s)
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO