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NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial (NDUFB8)
- Target
- NDUFB8
- Molecular classification
- Enzyme (accessory subunit, not catalytic), Mitochondrial protein, Component of respiratory chain Complex I
01
Overview
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial (NDUFB8), is an accessory (non-catalytic) subunit of the mitochondrial electron transport chain Complex I, the first and largest enzyme complex involved in oxidative phosphorylation. Located in the inner mitochondrial membrane, it plays a critical role in the assembly and stability of Complex I, facilitating transfer of electrons from NADH to ubiquinone to drive ATP synthesis. While not directly involved in catalysis, mutations or abnormal expression are associated with mitochondrial complex I deficiency and have been implicated as biomarkers in neurodegenerative diseases such as Alzheimer's and Parkinson's.
- Other names
- CI-ASHIComplex I-ASHINADH-ubiquinone oxidoreductase ASHI subunitNADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8NDUB8NDUB8_HUMANO95169
02
Mechanism of action
Drugs and toxins acting on mitochondrial Complex I (such as rotenone) inhibit transfer of electrons from NADH to ubiquinone, disrupting ATP synthesis and increasing oxidative stress, but do not target NDUFB8 specifically
03
Biological functions
Mitochondrial electron transportAssembly of respiratory chain Complex ICellular energy production (aerobic respiration)
04
Disease associations
Mitochondrial complex I deficiency, nuclear type 32Neurodegenerative disease (associated biomarker in Alzheimer's and Parkinson's disease)Atrial standstill 1
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Safety considerations
Inhibition or dysfunction of complex I, including loss of accessory subunits like NDUFB8, can cause or exacerbate mitochondrial disease, neurodegeneration, and energy metabolism disorders
06
Interacting drugs
No specific approved drugs directly target this subunit—however, several complex I inhibitors (e.g., rotenone, metformin, and others) target the entire complex rather than accessory subunits specifically
07
Biomarkers
Decreased expression or immunostaining of NDUFB8 is used as a biomarker for mitochondrial dysfunction, particularly in research on Alzheimer's disease, Parkinson's disease, and mitochondrial disorders
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