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HY Biochem Metab

Day 12.3

QuestionAnswer
Which glycogen storage disease matches the following phrase?: glycogen phosphorylase deficiency McArdle's disease (V)
Which glycogen storage disease matches the following phrase?: glucose-6-phosphatase deficiency Von Gierke's disease (I)
Which glycogen storage disease matches the following phrase?: lactic acidosis, hyperlipidemia, and hyperuricemia (gout) Von Gierke's disease (I)
Which glycogen storage disease matches the following phrase?: alpha-1,6-glucosidase deficiency Cori's disease (III)
Which glycogen storage disease matches the following phrase?: alpha-1,4-glucosidase deficiency Pompe's disease (II)
Which glycogen storage disease matches the following phrase?: cardiomegaly infantile Pompe's disease (II)
Which glycogen storage disease matches the following phrase?: diaphragm weakness--> respiratory failure adult Pompe's disease (II)
Which glycogen storage disease matches the following phrase?: increased glycogen in liver, severe fasting hypoglycemia Von Gierke's disease (I)
Which glycogen storage disease matches the following phrase?: heaptomegaly, hypoglycemia, hyperlipidemia (normal kidneys, lactate, and uric acid) Cori's disease (III)
Which glycogen storage disease matches the following phrase?: painful m cramps, myoglobinuria with strenuous exercise McArdle's disease (V)
Which glycogen storage disease matches the following phrase?: Severe hepatosplenomegaly, enlarged kidneys Von Gierke's disease (I)
Hemolytic anemia due to inability to maintain Na-K ATPase--> RBC swelling/lysis Glycolytic enzyme deficiency
Give the 2 MCC of glycolytic enzyme deficiency. Pyruvate kinase deficiency and phosphoglucose isomerase deficiency
A m biopsy on a pt of yours reveals elevated glycogen levels, elevated fructose-6-P, and decreased pyruvate. What enzyme deficiency do you suspect most? Deficiency in PFK-1
What enzymes are responsible for increasing and decreasing the intracellular levels of fru-2,6-bisP? PFK-2 increases levels and FBP-2 (fructose bisphosphatase-2) decreases levels
True or false: In the liver, high levels of glucagon result in elevated cAMP levels? True
True or false: Having an excess of fructose-2,6-bisphosphate results in more fructose-1,6-bisphosphate? True, by way of allosteric activation of PFK-1
How will levels of fructose-2,6-bisphosphate change in the liver and in the m during a sympathetic fight or flight response? Liver: levels will decrease (Less activation of PFK-1->less glycolysis) M: levels will increase (-> activation of PFK-1, sugar where you need it)
Why are the alanine and glutamine found in such high concentrations in the blood? They are two major carriers of nitrogen from tissues.
What is generally involved in transamination? Transfer of the amino group of an AA to alpha-ketoglutarate to form glutamate. The remaining deaminated AA is a keto-acid (such as pyruvate) that is used in energy metabolism.
What enzyme catalyzes transamination reactions? Aminotransferase
How are aminotransferases named? By the donor of the amino group (alanine aminotransferase converts alanine to pyruvate and forms glutamate).
In addition to substrates, what is required by all aminotransferases? Pyridoxal phosphate (B6). If you want to get rid of nitrogen, you need B6 to do it.
What are the two most important aminotransferase enzymes? What reaction do they catalyze? ALT and AST. *ALT: alanine + ketoglutarate-> glutamate + pyruvate. *AST: glutamate + oxaloacetate-> alpha-ketoglutarate + aspartate.
Name 3 substances that inhibit complex I. 1. Amytal (barbiturate) 2. Rotenone (fish poison) 3. MPP (derived MTPT)
Substance that inhibit complex III. Antimycin A
Substances that inhibit complex IV. 1. Hydrogen sulfide (H2S) 2. Carbon monoxide 3. Cyanide 4. Azide (N3-)
What fuels are produced in the post-absorptive period? Glucose (from liver glycogenolysis or gluconeogenesis) and FA/fatty acids (from adipose tissue)
Mm, brain, and other tissues predominantly use which fuel in the post-absorptive period? Glucose
When does gluconeogenesis begin in the post-absorptive period? Begins 4-6 hours after the last meal
When does gluconeogenesis become fully active in the post-absorptive period? Fully active when glycogen stores are depleted 10-18 hours after the last meal
How does the pattern of fuel production change in early starvation (24 hours after the last meal)? Glucose from gluconeogenesis and FA from adipose tissue
In early starvation (24 hours after the last meal), what fuel is primarily used by the brain? Glucose
In early starvation (24 hours after the last meal), what fuel is primarily used by mm and other tissues? FA (also use some glucose)
In intermediate starvation (48 hours after the last meal), how does the pattern of fuel production and consumption change? Glucose is produced from liver gluconeogenesis only (no more glycogenolysis), FA from adipose tissue, and ketone bodies from the liver
In the intermediate starvation period (48 hours after a meal), what fuel is predominantly used by the brain? Glucose (but also some ketone bodies)
In the intermediate starvation period (48 hours after a meal), what fuel is predominantly used by the mm and other tissues? FA (but also some ketone bodies)
What metabolic scenario favors the synthesis of ketone bodies? Excess acetyl-CoA from FA metabolism.
True or false? Ketone bodies can be used by all body tissues including the brain. False. RBCs can only use glucose
How is fuel produced in prolonged starvation (5 days after last meal)? Glucose from gluconeogenesis, FA from adipose tissue, ketone bodies from liver
In prolonged starvation (5 days after last meal), what fuel does the brain predominantly use? Ketone bodies
In prolonged starvation (5 days after last meal), what fuel do the mm and other tissues predominantly use? FA, but also some ketone bodies
During an overnight fast, what percentage of energy comes from glucose and what percentage comes from ketone bodies? 95% glucose and 5% ketones (majority of energy comes from glucose)
During a 3 day fast, what percentage of energy comes from glucose and what percentage comes from ketone bodies? 60% from ketone bodies and 40% from glucose (majority of energy comes from ketone bodies)
What are the major regulatory enzymes of the citric acid cycle? Citrate synthase (1st step), isocitrate dehydrogenase (RLS), alpha-ketoglutarate dehydrogenase (heavily regulated with lots of co-factors: TLC For No One)
What is the RLS for the following metabolic pathway: glycolysis PFK-1
What is the RLS for the following metabolic pathway: gluconeogenesis F-1,6-BPase
What is the RLS for the following metabolic pathway: citric acid cycle Isocitrate dehydrogenase
What is the RLS for the following metabolic pathway: glycogenesis Glycogen synthase
What is the RLS for the following metabolic pathway: glycogenolysis Glycogen phosphorylase
What is the functional role of S-adenosyl-methionine? Adds methyl group (generates phosphocreatinine as well)
What is the activated carrier for the following molecule?: CO2 biotin
What is the activated carrier for the following molecule?: glucose UDP glucose
What is the activated carrier for the following molecule?: Electrons NADH, NADPH, FADH2
What is the activated carrier for the following molecule?: one carbon units tetrahydrofolate
What is the activated carrier for the following molecule?: acyl coenzyme A and lipoamide
How many ATP are generated during aerobic metabolism? Malate-aspartate shuttle: 32 ATP Glycerol-3-P shuttle: 30 ATP
How many ATP are generated during anaerobic metabolism? 2 ATP (+ lactate molecule)
What are the possible products of pyruvate? Alanine, oxaloacetate, acetyl CoA, lactate/lactic acid
What irreversible enzymes are involved in gluconeogenesis? Pyruvate carboxylase, PEP carboxykinase, F-1,6-BPase (RLS), and G-6-Pase
What is the primary energy source in a patient that has not eaten in two days? Fat from adipose tissue
Arrange the following molecules from most exergonic with loss of phosphate to least exergonic with loss of phosphate: adenosine monophosphate, adenosine triphosphate, phosphoenolpyruvate. PEP > ATP > AMP
A stressed physician comes home from work, consumes 7 or 8 shots of tequila in rapid succession before dinner, and becomes hypoglycemic. Why? Because she was stressed out! Just kidding! B/c alcohol creates an excess of NADH which pushes pyruvate and OAA to make lactate and malate (respectively), so you can't enter gluconeogenesis.
A woman commonly develops intense m cramps and darkening of her urine after exercise. What is her diagnosis? McArdle's
Created by: sarah3148