The buildup of hydrogen is then use by the protein ATP synthase as a source of chemical potential energy to synthesise ATP from ADP and inorganic phosphate. The ADP is sent in from the cytoplasm while the ATP is sent out using a transport protein known as adenine nucleotide translocase. This complex process at the end of cellular respiration is crucial since it provides 32/36 ATP created by cellular respiration. In patience with CPEO, varying stages of ETC can be affected due to genetic mutations in nuclear DNA.
Importance of Mitochondria
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Oxidative phosphorylation is responsible for the synthesis of the majority of ATP (figure 1) which is used as the primary energy source of cells. The process begins when coenzymes of NADH and FADH2 reduced with hydrogen ions and electrons are transported by proteins to the inner mitochondrial membrane.
figure 1
ATP and Sources of Energy.pptx. (n.d.). Retrieved January 01, 2018, from https://www.emaze.com/@AORTCLRL/atp-and-sources-of-energypptx
The inner membrane is made of a phospholipid bilayer and encases the mitochondrial matrix with many folds and turns. This membrane is the site for the process where energy is transformed and transferred to synthesize ATP known as an electron transport chain or ETC.
The first site of electron transfer is between the NADPH to complex I and is transported to complex III, complex IV, and ends by binding to oxygen. The first complex is also where NADPH loses its hydrogen to later be transported by one of the three complex to the intermembrane space of the mitochondria. FADH2 has a similar path but it first interacts with complex II.