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26 Cards in this Set
- Front
- Back
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A deficiency of which glycolytic enzyme is associated with hemolytic anemia?
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pyruvate kinase - G6PDH is not part of glycolysis, it is part of the HMP shunt
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How do RBC's metabolize glucose?
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anaerobically (no mitochondria) thus depend soley on glycolysis
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Pyruvate dehydrogenase complex contains 3 enzymes that require 5 cofactors, what are they?
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1. Pyrophosphate (B1, thiamine, TPP) 2. FAD (B2, riboflavin) 3. NAD (B3, niacin) 4. CoA (B5, pantothenate) 5. Lipoic acid
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What are the only two purely ketogenic amino acids?
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Lysine and Leucine
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What factors increase the activation of the pyruvate dehydrogenase complex?
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high ADP ratio (exercise), high NAD/NADH ratio, high Ca+2
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Inhibition of lipoic acid leading to vomiting, rice water stools, and garlic breath is caused by what?
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Arsenic
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Pyruvate dehydrogenase deficiency results in ?
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backup of substrates resulting in lactic acidosis
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How is pyruvate dehydrogenase deficiency acquired?
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congenital or acquired (as in alcoholics due to B1 deficiency)
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Clinical findings in pyruvate dehydrogenase deficiency?
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Neurologic defects
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Treatment of pyruvate dehydrogenase deficiency?
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increase intake of ketogenic nutrients - high in fat content or lysine and leucine
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How many ATP equivalents are needed to generate glucose from pyruvate?
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6 ATP equivalents
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What are the 4 fates for pyruvate at the end of glycolysis?
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1. Alanine 2. oxaloacetate 3. Acetyl CoA 4. Lactate
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What is the function of the Cori cycle?
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transfers excess reducing equivalents from RBCs and muscle to the liver; shifts the metabolic burden to the liver
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What are the steps in the TCA cycle?
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Citrate > isocitrate > a-ketoglutarate > succinyl CoA > succinate > fumate > malate > OAA
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What cofactors are required for the a-ketoglutarate dehydrogenase complex?
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B1, B2, B3, B5, lipoic acid
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Which complexes bring protons across the inner mitochondrial membrane?
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Complexes I, III, IV
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In oxidative phosphorylation, how many ATP are produced from 1 NADH?
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3 ATP
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In oxidative phosphorylation, how many ATP are produced from 1 FADH2?
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2 ATP
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What are the three categories of oxidative phosphorylation poisons?
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1. electron transport inhibitors (Rotenone, CN-, Antimycin A, CO) 2. ATPase inhibitors (Oligomycin) 3. Uncoupling agents (UCP, 2,4-DNP, aspirin)
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What are the 4 irreversible enzymes of gluconeogenesis and where are they located?
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1. Pyruvate carboxylase (mitochondria) 2. PEP carboxykinase (PEPCK, cytosol) 3. Fructose 1,6-bisphosphatase (cytosol) 4. Glucose 6-phophotase (ER)
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What tissues contain the irreversible enzymes of gluconeogenesis?
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liver, kidney, intestinal epithelium - muscle does not contain G6Ptase and cannot participate in gluconeogenesis
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Deficiency of key gluconeogenic enzymes causes what symptoms?
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hypoglycemia
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The HMP shunt occurs in what parts of the body?
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lactating mammary glands, liver, adrenal cortex
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In what part of the cell does the HMP shunt occur?
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cytoplasm
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What enzyme is required for the irreversible reaction of the HMP shunt producing NADPH?
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Glucose-6-phosphate dehydrogenase
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What is the product of the reversible reaction of HMP shunt?
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Ribose-5-phosphate (for nucleotide synthesis) and Glyceraldehyde 3-phosphate, fructose 6-phosphate(intermediate of gyloslysis)
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