Target intelligence / Profile preview

NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (NDUFV2)

Target
NDUFV2
Molecular classification
Enzyme, Mitochondrial respiratory chain complex I core subunit, Oxidoreductase
01

Overview

NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (NDUFV2) is a core nuclear-encoded subunit of mitochondrial respiratory chain complex I, responsible for catalyzing electron transfer from NADH to ubiquinone, a critical step in cellular energy metabolism[1][2][4][6]. The protein contains a conserved iron-sulfur cluster ([2Fe-2S] N1a) and is localized to the inner mitochondrial membrane as part of the complex I peripheral arm[4][6]. NDUFV2 plays an essential role in electron transport and redox reactions. Mutations are linked to several diseases, including neurodegenerative and psychiatric disorders, and mitochondrial cardiomyopathies[1][4][7]. This subunit is indispensable for normal mitochondrial function and ATP production, making it a validated therapeutic target for conditions related to mitochondrial dysfunction[1][7].

Other names
NADH:ubiquinone oxidoreductase core subunit V2NADH-ubiquinone oxidoreductase 24 kDa subunitComplex I 24kDa subunitNADH dehydrogenase (ubiquinone) flavoprotein 2, mitochondrialNuclear-encoded mitochondrial NADH-ubiquinone reductase 24Kd subunitMC1DN7EC 7.1.1.2
02

Mechanism of action

Drugs aimed at NDUFV2 or complex I typically inhibit or modulate electron transfer, affecting mitochondrial metabolism and reactive oxygen species production.

03

Biological functions

Electron transfer in mitochondrial respiratory chainMitochondrial electron transport, NADH to ubiquinoneCardiac muscle tissue developmentNervous system developmentRedox/oxidoreductase activity
04

Disease associations

Neurodegenerative disease (Parkinson’s disease, Alzheimer’s disease)Psychiatric disorders (Bipolar disorder, Schizophrenia)Cardiovascular disease (hypertrophic cardiomyopathy)Mitochondrial complex I deficiencyProgressive cavitating leukoencephalopathyEncephalopathy
05

Safety considerations

Potential for mitochondrial toxicity/respiratory chain inhibition when modulating complex IRisk of exacerbating energetic failure or ROS production
06

Interacting drugs

NADH (nutraceutical; not a direct inhibitor or activator—no well-characterized approved drugs specifically target NDUFV2 as a molecular target[6])
07

Biomarkers

Genetic mutations in NDUFV2 (used for diagnosis and prognosis of certain mitochondrial disorders)Expression levels in mitochondrial diseases

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