A young animal has never had much energy. He is brought to a veterinarian for help and is sent to the animal hospital for some tests. There they discover his mitochondria can use only fatty acids and amino acids for respiration, and his cells produce more lactate than normal. Of the following, which is the best explanation of his condition?
A) His mitochondria lack the transport protein that moves pyruvate across the outer mitochondrial membrane. B) His cells cannot move NADH from glycolysis into the mitochondria. C) His cells contain something that inhibits oxygen use in his mitochondriaD) His cells lack the enzyme in glycolysis that forms pyruvate.
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Ответ:
A. His mitochondria lack the transport protein that moves pyruvate across the outer mitochondrial membrane.
Explanation:
Pyruvate is from the breakdown of carbohydrates such as glucose through glycolysis. Glucose enters the cytosol through specific transporters (the GLUT family) and is processed by one of several pathways depending on cellular requirements. Glycolysis occurs in the cytosol and produces a limited amount of ATP, but the end product is two 3-carbon molecules of pyruvate, which maybe diverted again into many pathways depending on the requirements of the cell. In aerobic conditions, pyruvate is primarily transported into the mitochondrial matrix and converted to acetyl-coenzyme A (acetyl-CoA) and carbon dioxide by the pyruvate dehydrogenase complex (PDC).
Initially it was proposed that pyruvate was able to cross the membrane in its undissociated (acid) form but evaluation of its biochemical properties show that it is largely in its ionic form within the cell and should therefore require a transporter.
Transport of pyruvate across the outer mitochondrial membrane appears to be easily accomplished via large non-selective channels such as voltage-dependent anion channels/porin, which enable passive diffusion. Indeed, deficiencies in these channels have been suggested to block pyruvate metabolism
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