Recombinant Lagostrophus fasciatus NADH-ubiquinone oxidoreductase chain 4L (MT-ND4L)
Description
Functional Role in Complex I
As a subunit of Complex I, MT-ND4L contributes to:
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Electron transport: Transfers electrons from FMNH₂ to iron-sulfur clusters and ubiquinone .
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Proton pumping: Generates a proton gradient across the mitochondrial membrane, driving ATP synthesis .
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Complex assembly: Interacts with mitochondrial-encoded and nuclear-encoded subunits to stabilize Complex I’s structure .
Disruption of MT-ND4L function, as observed in human MT-ND4L mutations, is linked to Leber’s Hereditary Optic Neuropathy (LHON) and metabolic disorders .
Research Applications and Challenges
Recombinant MT-ND4L proteins are used to:
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Study Complex I assembly: Investigate subunit interactions and electron transport mechanisms .
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Model mitochondrial diseases: Analyze mutations linked to LHON and metabolic disorders .
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Develop diagnostic tools: Enable functional assays for Complex I activity .
Challenges include:
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Low solubility: Hydrophobic nature complicates purification and structural studies .
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Species-specific variability: Sequence divergence may affect cross-species functional studies .
Clinical and Biological Significance
MT-ND4L dysfunction is implicated in:
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Leber’s Hereditary Optic Neuropathy: A T10663C mutation in human MT-ND4L causes optic nerve degeneration .
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Metabolic disorders: Variants correlate with obesity, diabetes, and hypertension .
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Neuroprotection: Overexpression of NDUFA4 (a Complex I subunit) promotes neuronal survival via anti-apoptotic pathways .
Product Specs
Note: We will prioritize shipping the format currently in stock. However, if you have specific format requirements, please indicate them in your order. We will fulfill your request to the best of our ability.
Note: All our proteins are shipped with standard blue ice packs. If you require dry ice shipping, please inform us in advance. Additional fees may apply.
