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HY Biochem Metab
Day 12.3
| Question | Answer |
|---|---|
| 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
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