Investigating Secondary Oxidative Phosphorylation Complex I Defects in Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha Deficiency

The mitochondrion is the 'powerhouse' of a cell that produces energy in the form of adenosine triphosphate (ATP). The Tricarboxylic acid cycle (TCA), fatty acid ß-oxidation (FAO), and oxidative phosphorylation (OXPHOS) are the three main biochemically interlinked pathways that collaboratively produce ATP in eukaryotic cells, and their proteins and complexes often interact with each other.