NDUFA6
Description
The NDUFA6 (NADH:ubiquinone oxidoreductase subunit A6) is a protein-coding gene located on chromosome 22.
The NDUFA6 gene, located on chromosome 22, encodes for NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6. This protein, weighing 18 kDa, is a subunit of the NADH dehydrogenase (ubiquinone) enzyme, which is the largest of the five complexes in the electron transport chain within the mitochondrial inner membrane. The gene spans 5,359 base pairs and its structure includes a hydrophobic transmembrane domain and a hydrophilic domain. The N-terminal hydrophobic domain is predicted to form an alpha helix that spans the inner mitochondrial membrane, while the C-terminal hydrophilic domain interacts with the globular subunits of Complex I. The conserved two-domain structure is likely essential for protein function. NDUFA6 is one of approximately 31 hydrophobic subunits that contribute to the transmembrane region of Complex I. Its secondary structure is primarily alpha helix, but the carboxy-terminal half has the potential to form a coiled-coil. The amino-terminal region contains a beta sheet rich in hydrophobic amino acids, potentially serving as a mitochondrial import signal. Related pseudogenes have also been identified on four other chromosomes.
NDUFA6 is also known as B14, CI-B14, LYRM6, MC1DN33, NADHB14.
