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NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 (NDUFA5)
- Target
- NDUFA5
- Molecular classification
- Enzyme (respiratory chain accessory subunit), Mitochondrial complex I protein
01
Overview
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 (NDUFA5) is a nuclear-encoded accessory subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase), which is located in the inner mitochondrial membrane and participates in the transfer of electrons from NADH to ubiquinone as part of the respiratory chain. While NDUFA5 is not directly involved in the catalytic activity of complex I, it is essential for complex stability and function, contributing to mitochondrial energy production via oxidative phosphorylation[1][5][6]. Mutations or dysregulation of NDUFA5 have been associated with specific mitochondrial disorders and have downstream effects on cellular metabolism[1][5].
- Other names
- Complex I 13kDa subunit BComplex I subunit B13Ubiquinone reductaseType I dehydrogenaseNADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5 (13kDa, B13)Complex I-13kD-BCI-13kD-BCI-13kBB13NUFMUQOR13
02
Mechanism of action
Drugs that inhibit mitochondrial complex I (e.g., rotenone) inhibit the entire complex, not specifically NDUFA5
03
Biological functions
Electron transportMitochondrial respirationOxidative phosphorylation
04
Disease associations
Mitochondrial diseasePossibly neurodegenerative disease (by virtue of core complex I involvement)Other rare diseases (e.g., endobronchial leiomyoma)
05
Safety considerations
Targeting this protein may disrupt mitochondrial function and cellular energy metabolismLoss of function could cause or worsen mitochondrial disorders
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