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(BQ) Contniued part 1, part 2 of the document Biochemistry essential has contents: Oxidative phosphorylation and mitochondrial diseases, glycogen metabolism and glycogen storage diseases, gluconeogenesis and fasting hypoglycemia, insulin and counterregulatory hormones,. and other contents. Invite you to refer. | Chapter 23 Oxidative Phosphorylation and Mitochondrial Diseases SYNOPSIS Mitochondria are basically stripped-down gram-negative bacteria that specialize in energy production. Human mitochondria consist of an internal compartment the mitochondrial matrix that contains the enzymes of the citric acid cycle fatty acid p-oxidation ketone body metabolism and parts of several biosynthetic pathways. The matrix is enclosed by the inner mitochondrial membrane which contains the proteins for oxidative phosphorylation. The inner mitochondrial membrane is surrounded by an outer mitochondrial membrane that is permeable to small molecules. The region between the two membranes is the intermembrane space. Oxidative phosphorylation takes place in the mitochondria and couples the oxidation of reduced nicotinamide adenine dinucleotide NADH and other reduced compounds to the production of adenosine triphosphate ATP Fig. 23.1 . As NADH is oxidized protons H are pumped out of the matrix into the intermembrane space as part of a series of oxidation-reduction reactions. An ATP synthase allows protons to flow back into the mitochondrial matrix and it uses the energy that is freed in this process to phosphorylate adenine diphosphate ADP to ATP. Mitochondria contain their own DNA. Mitochondria are inherited from only the mother. Some of the proteins needed for oxidative phosphorylation are encoded by the DNA in the mitochondria but most are derived from the DNA in the nucleus. Mitochondrial diseases give rise to deficient oxidative phosphorylation and consequently affect primarily cells and tissues that require a high rate of ATP production such as the central nervous system the heart and skeletal muscle. Pancreatic p -cells are also often affected since ATP synthesis is required for glucose sensing and insulin secretion. LEARNING OBJECTIVES For mastery off this topic you should be able to do the -following Describe the function cellular location and tissue distribution of the electron .
