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TPI deficiency is a rare human condition. Patients who lack TPI cannot convert the triose dihydroxyacetone phosphate into glyceraldehyde 3-phosphate. What happens to glycolysis in TPI patients? TPI patients suffer from chronic hemolytic anemia (abnormal breakdown of red blood cells) and have variable neuromuscular dysfunctions, including muscle weakness, poor muscle tone, and atrophy. Most die of respiratory failure during childhood. Explain why TPI-deficient patients exhibit these conditions using the diagram to justify your answer. TPI is enzyme 5.

TPI deficiency is a rare human condition. Patients who lack TPI cannot convert the triose dihydroxyacetone phosphate into glyceraldehyde 3-phosphate. What happens to glycolysis in TPI patients? TPI patients suffer from chronic hemolytic anemia (abnormal breakdown of red blood cells) and have variable neuromuscular dysfunctions, including muscle weakness, poor muscle tone, and atrophy. Most die of respiratory failure during childhood. Explain why TPI-deficient patients exhibit these conditions using the diagram to justify your answer. TPI is enzyme 5.

Biochemistry
Biochemistry
9th Edition
ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Chapter1: Biochemistry: An Evolving Science
Section: Chapter Questions
Problem 1P
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TPI deficiency is a rare human condition. Patients who lack TPI cannot convert the triose
dihydroxyacetone phosphate into glyceraldehyde 3-phosphate.
What happens to glycolysis in TPI patients?

TPI patients suffer from chronic hemolytic anemia (abnormal breakdown of red blood cells)
and have variable neuromuscular dysfunctions, including muscle weakness, poor muscle tone,
and atrophy. Most die of respiratory failure during childhood.
Explain why TPI-deficient patients exhibit these conditions using the diagram to justify your
answer. TPI is enzyme 5.

G6PD deficiency is one of the most common human genetic conditions, affecting about 40 million
people worldwide. Patients suffer from hemolytic anemia when exposed to certain drugs, viral or
bacterial disease, or fava beans.

HANDOUT A Glucose-6 Phosphate Dehydrogenase and TPI Deficiencies OH OH 2-O,PO-CH, OH O *OPO—CH, CH,OH OH Mutase OH OH 2-0,PO-CH₂ Q OH 2,3-Bisphosphoglycerate Phosphatase OH HỌ 2,3-Bisphosphoglycerate Bisphosphoglycerate НО OH OH CH,OH OH CH₂-OPO,²- OH ATP+ C-OPO,²- CH₂ Glucose ATP HC=O HC-OH Glyceraldehyde-3-P H₂C-OPO,²- P NAD NADH H₂O+ Glucose-6-P ATP Fructose-6-P◄ 1,3-Bisphosphoglycerate Fructose-1,6-BP 1 Hexokinase ATP 3 Phosphofructokinase-1 -O HC OH 3-Phosphoglycerate H₂C-OPO,² Glucose-6-Phosphate Dehydrogenase 2 Phosphoglucoisomerase Pyruvate Triose H₂C-OH Phosphate Dihydroxyacetone-P C=O Isomerase Glyceraldehyde-3-P dehydrogenase 2-Phosphoglycerate NADP NADPH A 4 Aldolase Phosphoglycerate kinase Phosphoenolpyruvate Phosphoglycerate mutase 8 Enolase 6-phosphoglucolactone. Pyruvate kinase Non-oxidative Phase of Pentose Phosphate Pathway H₂C-OPO,¹ 0=000 -OPO,²- HC-OH H₂C-OPO,¹ 요。 HC-OPO, H₂C-OH c=o CH₂ H₂O H 6-Phosphoglucolactonase 6-phosphogluconate 6-Phosphogluconate Dehydrogenase NADP+ NADPH CO₂ Ribulose-5-Phosphate The diagram above shows two shunts from glycolysis. The first, the oxidative pentose phosphate shunt (or the hexose monophosphate pathway, HMP), generates five carbon sugars (pentoses) and NADPH. The pentoses are used in nucleotide synthesis reactions, and the NADPH is used is in fatty acid synthesis and in reduction reactions. The reduction reactions are important in red blood cells to help reduce oxidative stresses on the cell membranes. The second pathway, the Rapoport-Luebering glycolytic shunt, produces 2,3-bisphosphoglycerate, which helps to displace oxygen in red blood cells; this aids in oxygen delivery to the tissues. At least two human conditions affect glycolytic pathways: glucose 6-P dehydrogenase (G6PD) deficiency and triose phosphate isomerase (TPI) deficiency. G6PD deficiency is an X-linked disease, while the TPI gene is found on chromosome 12.
Transcribed Image Text:HANDOUT A Glucose-6 Phosphate Dehydrogenase and TPI Deficiencies OH OH 2-O,PO-CH, OH O *OPO—CH, CH,OH OH Mutase OH OH 2-0,PO-CH₂ Q OH 2,3-Bisphosphoglycerate Phosphatase OH HỌ 2,3-Bisphosphoglycerate Bisphosphoglycerate НО OH OH CH,OH OH CH₂-OPO,²- OH ATP+ C-OPO,²- CH₂ Glucose ATP HC=O HC-OH Glyceraldehyde-3-P H₂C-OPO,²- P NAD NADH H₂O+ Glucose-6-P ATP Fructose-6-P◄ 1,3-Bisphosphoglycerate Fructose-1,6-BP 1 Hexokinase ATP 3 Phosphofructokinase-1 -O HC OH 3-Phosphoglycerate H₂C-OPO,² Glucose-6-Phosphate Dehydrogenase 2 Phosphoglucoisomerase Pyruvate Triose H₂C-OH Phosphate Dihydroxyacetone-P C=O Isomerase Glyceraldehyde-3-P dehydrogenase 2-Phosphoglycerate NADP NADPH A 4 Aldolase Phosphoglycerate kinase Phosphoenolpyruvate Phosphoglycerate mutase 8 Enolase 6-phosphoglucolactone. Pyruvate kinase Non-oxidative Phase of Pentose Phosphate Pathway H₂C-OPO,¹ 0=000 -OPO,²- HC-OH H₂C-OPO,¹ 요。 HC-OPO, H₂C-OH c=o CH₂ H₂O H 6-Phosphoglucolactonase 6-phosphogluconate 6-Phosphogluconate Dehydrogenase NADP+ NADPH CO₂ Ribulose-5-Phosphate The diagram above shows two shunts from glycolysis. The first, the oxidative pentose phosphate shunt (or the hexose monophosphate pathway, HMP), generates five carbon sugars (pentoses) and NADPH. The pentoses are used in nucleotide synthesis reactions, and the NADPH is used is in fatty acid synthesis and in reduction reactions. The reduction reactions are important in red blood cells to help reduce oxidative stresses on the cell membranes. The second pathway, the Rapoport-Luebering glycolytic shunt, produces 2,3-bisphosphoglycerate, which helps to displace oxygen in red blood cells; this aids in oxygen delivery to the tissues. At least two human conditions affect glycolytic pathways: glucose 6-P dehydrogenase (G6PD) deficiency and triose phosphate isomerase (TPI) deficiency. G6PD deficiency is an X-linked disease, while the TPI gene is found on chromosome 12.
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