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Target intelligence / Profile preview
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NDUFB11)
- Target
- NDUFB11
- Molecular classification
- Enzyme (Complex I accessory subunit), Mitochondrial respiratory chain complex I component
01
Overview
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NDUFB11), is an accessory subunit of mitochondrial respiratory chain complex I, located on the inner mitochondrial membrane. While not directly involved in catalysis, it contributes to the assembly and stability of complex I, which plays a critical role in transferring electrons from NADH to ubiquinone in the process of oxidative phosphorylation. Mutations in NDUFB11 are associated with mitochondrial complex I deficiency and congenital syndromes involving multi-organ developmental defects[1][5][6].
- Other names
- ESSSNP17.3Np15NADH-ubiquinone oxidoreductase ESSS subunitNADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDaNeuronal protein 17.3Complex I-ESSS
02
Mechanism of action
No drugs directly target NDUFB11; drugs targeting mitochondrial respiratory chain (complex I) inhibit the electron transfer function (general mechanism for complex I inhibitors).
03
Biological functions
Mitochondrial electron transport chainOxidative phosphorylationTransfer of electrons from NADH to ubiquinone
04
Disease associations
Mitochondrial complex I deficiencyLinear skin defects with multiple congenital anomaliesCardiomyopathyPotential role in atherosclerosis and stress response
05
Safety considerations
Mutations may result in multi-systemic disorders, including lethal cardiomyopathy, neurodevelopmental defects, and congenital anomalies[1][5]
06
Biomarkers
Deficiency or mutation is a biomarker for mitochondrial complex I deficiency and associated syndromes[1][5]
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