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19 Nov 2019
Following is a list of hereditary metabolic defects involving loss of single enzymes of catabolism, and a second list of possible consequences of such defects. Match each enzyme defect with its most likely consequence (only one) from the second list. Defects: Lack of pyridoxal kinase (catalyzes conversion of pyridoxal to pyridoxal phosphate) Lack of propionyl CoA carboxylase (conversion of propionyl CoA to succinyl CoA) Lack of isocitrate dehydrogenase Lack of oxaloacetate decarboxylase Lack of pyruvate dehydrogenase Consequences: Impaired ability to obtain energy from short-chain fatty acids of odd carbon number, with little if any effect on ability to obtain energy from proteins Impaired ability to obtain energy from all fatty acids Impaired ability to obtain energy from proteins, with little if any effect on ability to obtain energy from carbohydrate Inability to synthesize or degrade almost all amino acids Impaired ability to obtain energy from proteins, and inability to obtain energy from carbohydrate Impaired ability to excrete amino acid nitrogen Lethal; prevents complete oxidation of all fuel molecules
Following is a list of hereditary metabolic defects involving loss of single enzymes of catabolism, and a second list of possible consequences of such defects. Match each enzyme defect with its most likely consequence (only one) from the second list. Defects: Lack of pyridoxal kinase (catalyzes conversion of pyridoxal to pyridoxal phosphate) Lack of propionyl CoA carboxylase (conversion of propionyl CoA to succinyl CoA) Lack of isocitrate dehydrogenase Lack of oxaloacetate decarboxylase Lack of pyruvate dehydrogenase Consequences: Impaired ability to obtain energy from short-chain fatty acids of odd carbon number, with little if any effect on ability to obtain energy from proteins Impaired ability to obtain energy from all fatty acids Impaired ability to obtain energy from proteins, with little if any effect on ability to obtain energy from carbohydrate Inability to synthesize or degrade almost all amino acids Impaired ability to obtain energy from proteins, and inability to obtain energy from carbohydrate Impaired ability to excrete amino acid nitrogen Lethal; prevents complete oxidation of all fuel molecules
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