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NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9 (NDUFA9)
- Target
- NDUFA9
- Molecular classification
- Accessory subunit, Mitochondrial protein, Complex I component
01
Overview
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9 (NDUFA9) is an accessory subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase). It is crucial for the proper assembly and structural integrity of Complex I, which is a key component of the electron transport chain. While NDUFA9 is not directly involved in catalysis, it stabilizes the junction between the membrane and matrix arms of Complex I. Mutations in NDUFA9 or disruptions in its interactions can lead to Complex I dysfunction, associated with severe neurodegenerative diseases like Leigh syndrome.
- Other names
- CI-39kDComplex I-39kDCC6CI-39kCI39kNDUFS2LSDR22E1MC1DN26COQ11
02
Mechanism of action
N/A (structural protein)
03
Biological functions
Stabilization of Complex IComplex I assemblyStructural integrity of Complex IElectron transport (indirectly)
04
Disease associations
Leigh SyndromeMitochondrial complex I deficiencyNeurodegenerative diseases
05
Safety considerations
Complex I dysfunctionMitochondrial toxicity (indirect)
06
Biomarkers
NDUFA9 expression levels (potential)Complex I activity (indirect)
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