Target intelligence / Profile preview

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial (NDUFA10)

Target
NDUFA10
Molecular classification
Enzyme (specifically, mitochondrial complex I accessory subunit), Mitochondrial membrane respiratory chain component
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Overview

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial (NDUFA10), is an accessory subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase), which is the first and largest enzyme of the mitochondrial respiratory electron transport chain. NDUFA10 is believed not to be directly involved in catalysis but is required for proper complex I assembly or stability. Its principal function is part of the multi-protein machinery that transfers electrons from NADH to ubiquinone, coupled to proton translocation, driving ATP synthesis by oxidative phosphorylation. Mutations in NDUFA10 have been implicated in severe mitochondrial disorders, including Leigh syndrome and other forms of mitochondrial complex I deficiency.

Other names
NDUFA10NADH:ubiquinone oxidoreductase subunit A10CI-42kDCI-42kComplex I-42kDNADH-ubiquinone oxidoreductase 42 kDa subunitcomplex I 42kDa subunitCI-42KDMC1DN22
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Mechanism of action

For the overall complex: facilitation of electron transfer from NADH to ubiquinone, contributing to oxidative phosphorylation; NDUFA10 itself believed to be an accessory subunit not directly involved in catalysis

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Biological functions

Electron transport in the mitochondrial respiratory chainOxidoreductase activityNADH dehydrogenase activity (transfers electrons from NADH to ubiquinone in the respiratory chain)
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Disease associations

Mitochondrial complex I deficiencyLeigh syndromeOther mitochondrial disorders
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Safety considerations

Mutations can cause severe mitochondrial dysfunction, leading to fatal or disabling syndromes (not directly drug-targetable safety issues, rather genetic disease complications)
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Biomarkers

Mutations may be used for diagnosing specific forms of mitochondrial complex I deficiency or Leigh syndrome

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