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NDUFA6

Protein-coding gene in the species Homo sapiens

Protein-coding gene in the species Homo sapiens

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 is an enzyme that in humans is encoded by the NDUFA6 gene. It belongs to the superfamily of LYRM proteins, which are characterized by a conserved leucine–tyrosine–arginine motif. The NDUFA6 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain.

Structure

The NDUFA6 gene is located on the q arm of chromosome 22 in position 13.2 and spans 5,359 base pairs. The gene produces an 18 kDa protein composed of 154 amino acids. NDUFA6 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes. The structure is L-shaped with a long, hydrophobic transmembrane domain and a hydrophilic domain for the peripheral arm that includes all the known redox centers and the NADH binding site.

Function

The human NDUFA6 gene codes for a subunit of Complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. Initially, NADH binds to Complex I and transfers two electrons to the isoalloxazine ring of the flavin mononucleotide (FMN) prosthetic arm to form FMNH2. The electrons are transferred through a series of iron-sulfur (Fe-S) clusters in the prosthetic arm and finally to coenzyme Q10 (CoQ), which is reduced to ubiquinol (CoQH2). The flow of electrons changes the redox state of the protein, resulting in a conformational change and pK shift of the ionizable side chain, which pumps four hydrogen ions out of the mitochondrial matrix.

References

References

  1. "Entrez Gene: NDUFA6 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6".
  2. (May 2024). "Installation of LYRM proteins in early eukaryotes to regulate the metabolic capacity of the emerging mitochondrion". Open Biology.
  3. Donald Voet. (2013). "Fundamentals of biochemistry : life at the molecular level". Wiley.
  4. (Oct 2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research.
  5. (July 2025). "NDUFA6 - NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 }}{{Dead link". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB).
  6. (Nov 1998). "Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chain". Cytogenet Cell Genet.
  7. (Nov 1998). "Mapping of the NDUFA2, NDUFA6, NDUFA7, NDUFB8, and NDUFS8 electron transport chain genes by intron based radiation hybrid mapping". Cytogenet Cell Genet.
  8. (Jan 1998). "Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits". Biochem Biophys Res Commun.
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