The oxidative decarboxylation of Pyruvate is carried out by the pyruvate dehydrogenase complex. This multi-protein complex is composed of three enzymes; 1) pyruvate dehydrogenase, 2) dihdrolipoyl transacetylase, and 3) dihyrolipoyl dehydrogenase. The intermediate products formed by this complex are not released and remain bound to the enzyme. The breakdown of fatty acids by the β-oxidation pathway is carried out by four separate enzymes found in the mitochondrial matrix; 1) fatty acyl-CoA dehydrogenase, 2) enoyl hydratase, 3) β-hydroxyacyl-CoA dehydrogenase 4) β-ketoacyl-CoA thiolase. Each cycle of the pathway shortens the fatty acyl-CoA chain by two carbon units. This process continues until completed, resulting in the fatty acid being completely …show more content…
Human mitochondrial diseases affect people of all ages and can infect any organ in the body. To date, there is no cure for mitochondrial diseases, however there are treatments available to reduce the symptoms and the suffering of those infected. Mitochondrial disease arise when mutations occur in either mt DNA or nuclear genes that encode the mitochondrial proteins essential for the generation of ATP. Furthermore, recent research shows, that dysfunctional mitochondria in one tissue can have an effect on the whole organism (Nunnari & Suomalainen, …show more content…
As a result of the generation of mitochondrial (ROS) we see oxidative changes to proteins such as β-amyloid protein (Aβ) which may result in protein misfolding or lead to further interaction with mitochondria causing greater mitochondria dysfunction. Parkinsons’s Disease is another neurodegenerative disease. It is an incurable, progressive brain disorder which affects the nervous system causing a slowness in movement and an involuntary shaking. It is identified by the accumulation of nerve fibers in the brain and fibrous protein deposits in the neuronal cytoplasm. Recent studies have shown (Guo et al, 2013), that mutations in mitochondrial genes are implicated in Parkinsons. A reduction of activity of Complex I of the electron transport chain and mutations in mtDNA-encoded complex I subunits and mtDNA polymerase γ are associated with Parkinson’s. Huntington’s Disease is an inherited disorder which causes death of brain cells. Studies show that it is caused by the reduction of activity of complex II in the electron transport