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Huntington's disease (HD) is an autosomal dominant, 12 neurodegenerative disorder caused by a pathological expansion of CAG repeats in the gene encoding for a protein called huntingtin. CAG codes for glutamine, and huntingtin protein containing 2 42 ex- tra C-terminal Gin residues causes pathology. There has been sig- nificant evidence that energy production is impaired in HD, but the origin of the hypometabolism, i.e., glycolysis vs. oxidative phosphor- ylation, was not established until fairly recently. wild-type 10 mutant 8 2 The diagram on the right compares in the upper diagram the total ATP production generated in cultured (wt) STHDHQ7/Q7 and mu- tant STHDHQ111/Q111 striatal neuronal progenitor cells. Within the standard error of the measurements, the ATP production proved un- expectedly to be equivalent from both types of cells. To probe the two different sources of ATP production in the cells, the investigators 25 wild-typ mutant 20 15 carried out similar experiments with permeabilized cells metabolizing

Huntington's disease (HD) is an autosomal dominant, 12 neurodegenerative disorder caused by a pathological expansion of CAG repeats in the gene encoding for a protein called huntingtin. CAG codes for glutamine, and huntingtin protein containing 2 42 ex- tra C-terminal Gin residues causes pathology. There has been sig- nificant evidence that energy production is impaired in HD, but the origin of the hypometabolism, i.e., glycolysis vs. oxidative phosphor- ylation, was not established until fairly recently. wild-type 10 mutant 8 2 The diagram on the right compares in the upper diagram the total ATP production generated in cultured (wt) STHDHQ7/Q7 and mu- tant STHDHQ111/Q111 striatal neuronal progenitor cells. Within the standard error of the measurements, the ATP production proved un- expectedly to be equivalent from both types of cells. To probe the two different sources of ATP production in the cells, the investigators 25 wild-typ mutant 20 15 carried out similar experiments with permeabilized cells metabolizing

Human Anatomy & Physiology (11th Edition)
Human Anatomy & Physiology (11th Edition)
11th Edition
ISBN:9780134580999
Author:Elaine N. Marieb, Katja N. Hoehn
Publisher:Elaine N. Marieb, Katja N. Hoehn
Chapter1: The Human Body: An Orientation
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1. Huntington's disease (HD) is an autosomal dominant, 12 neurodegenerative disorder caused by a pathological expansion of CAG repeats in the gene encoding for a protein called huntingtin. CAG codes for glutamine, and huntingtin protein containing > 42 ex- tra C-terminal Gln residues causes pathology. There has been sig- nificant evidence that energy production is impaired in HD, but the origin of the hypometabolism, i.e., glycolysis vs. oxidative phosphor- ylation, was not established until fairly recently. wild-type 10 mutant 8 2 The diagram on the right compares in the upper diagram the total ATP production generated in cultured (wt) STHDHQ7/Q7 and mu- tant STHDHQ111/Q111 striatal neuronal progenitor cells. Within the standard error of the measurements, the ATP production proved un- expectedly to be equivalent from both types of cells. To probe the two different sources of ATP production in the cells, the investigators carried out similar experiments with permeabilized cells metabolizing succinate + rotenone or glutamate + malate in the presence of 2- deoxyglucose, as shown in the lower diagram. The resting respira- tory rate of permeabilized cells was first measured followed by addi- tion of ADP. The results showed that mitochondrial ATP production was deficient in the mutant cells. 25 wild-type mutant 20 15 10 succ.+rot. glut.+mal. (a) tion in the mutant HD mitochondria. Write the structural formula of 2-deoxyglucose either in its cyclic or linear form. Explain why the addition of 2-deoxyglucose revealed deficient mitochondrial ATP produc- (b) Write the reactions with structural formulas (except for nucleotides); name substrates, products, en- zymes, and cofactors. Which glycolytic reactions generating ATP are blocked in the presence of 2-deoxyglucose? nmols/min/mg protein ATP (nmols/mg)
Transcribed Image Text:1. Huntington's disease (HD) is an autosomal dominant, 12 neurodegenerative disorder caused by a pathological expansion of CAG repeats in the gene encoding for a protein called huntingtin. CAG codes for glutamine, and huntingtin protein containing > 42 ex- tra C-terminal Gln residues causes pathology. There has been sig- nificant evidence that energy production is impaired in HD, but the origin of the hypometabolism, i.e., glycolysis vs. oxidative phosphor- ylation, was not established until fairly recently. wild-type 10 mutant 8 2 The diagram on the right compares in the upper diagram the total ATP production generated in cultured (wt) STHDHQ7/Q7 and mu- tant STHDHQ111/Q111 striatal neuronal progenitor cells. Within the standard error of the measurements, the ATP production proved un- expectedly to be equivalent from both types of cells. To probe the two different sources of ATP production in the cells, the investigators carried out similar experiments with permeabilized cells metabolizing succinate + rotenone or glutamate + malate in the presence of 2- deoxyglucose, as shown in the lower diagram. The resting respira- tory rate of permeabilized cells was first measured followed by addi- tion of ADP. The results showed that mitochondrial ATP production was deficient in the mutant cells. 25 wild-type mutant 20 15 10 succ.+rot. glut.+mal. (a) tion in the mutant HD mitochondria. Write the structural formula of 2-deoxyglucose either in its cyclic or linear form. Explain why the addition of 2-deoxyglucose revealed deficient mitochondrial ATP produc- (b) Write the reactions with structural formulas (except for nucleotides); name substrates, products, en- zymes, and cofactors. Which glycolytic reactions generating ATP are blocked in the presence of 2-deoxyglucose? nmols/min/mg protein ATP (nmols/mg)
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