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Biochemistry 2280A Study Guide - Quiz Guide: Glycogen Phosphorylase, Glycogen Synthase, Atp Synthase

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oliveeagle385
15 Jan 2017
School
Western University
Department
Biochemistry
Course
Biochemistry 2280A
Professor
Dr.Mc Lachlin

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Document Summary

Blood glucose is an immediately available energy source for the cells as it is able to easily enter the cell by facilitated diffusion. It is available to the body as well as the brain since it is able to transverse the blood-brain-barrier. It is imported from the blood into cells, where it is then catabolized by glycolysis to produce pyruvate, atp, and nadh. Pyruvate is oxidized by the cell in the citric acid cycle to produce more electron carriers (nadh and fadh2) and more. Electron carriers are then sent to the inner membrane of the mitochondria where the electron transport chain produces energy in the form of atp. High levels of blood glucose activate insulin, which signals the liver to synthesize glycogen and signals cells to uptake glucose. Low levels of blood glucose activate glucagon, which signals cells to breaks down glycogen.

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Related Questions

(BIOCHEMISTRY QUESTION)

A. Gluconeogenesis is not just the reverse of glycolysis. Briefly describe one of the steps catalyzed by different enzymes in glycolysis and gluconeogenesis.

B. Explain how a biochemist might dicover that a certain enzyme is allosterically regulated.

C. What are the regulatory implications for the cell with regard to ATP & AMP, given the relative concentrations of ATP & AMP in a cell?

D. Describe the function of fructose 2,6-bisphosphate in overriding the control of gycolysis and gluconeogenesis in liver cells in response to lowered blood glucose levels.

E. The human disease beriberi is caused by a deficiency of thiamine in the diet. People with severe beriberi have higher than normal levels of pyruvate in their blood & urine. Explain this observation in terms of specific enzymatic reaction(s).

My Responses: Could you tell me if these are correct? If not, could you provide the correct answer and explanation?

A. Its a cellular process in which glucose is produced from pyruvate, amino acids, lactate, and other biogenic precursors.

B. They might learn that an enzyme is allosterically regulated if that protein or enzyme undergoes a conformational change modulated by the binding of one or more modulators/effectors to alternate sites on the protein, resulting in a change in its activity. And they usually have more than one polypeptide chain.

C. The regulatory implications for the cell would be that all activity would increase and concentrations would go up unless bound to PFK-1 and then the overall activity would drop.

D. Low blood glucose is when glucagon signals the liver to produce and release more glucose (gluconeogenesis) and stop consumption. Fructose 2, 6-bisphosphate mediates rapid hormonal regulation of glycolysis and gluconeogenesis.

E. Pyruvate is the product of both carbohydrate and amino acid catabolism that enters cellular respiration in the mitochondria when converted to acetyl-CoA catalyzed by the PDH complex. The first step in the PDH complex reaction is a decarboxylation reaction that cannot be catalyzed without the enzyme thiamine pyrophosphate (TPP). This coenzyme is a derivative of thiamine or vitamin B1.

1)Pyruvate is one of the products of glycolysis that can be used by aerobic organisms to extract additional energy. Explain what happens to pyruvate after glycolysis, name the multi-enzyme complex that catalyzes this reaction, and name the cycle that the product feeds into.

2)Suppose you start with 1 molecule of glucose and go through glycolysis to produce 2 molecules of pyruvate. These 2 molecules of pyruvate are then converted into acetyl CoA which then go through the TCA cycle. Theoretically, how many total ATP can be formed through this process? (Account for NADH/FADH2 produced). Show where each molecule of ATP is generated from.

3)Name the five types of membrane-bound electron carriers. All of the redox centers within the respiratory chain that accept and donate electrons are prosthetic groups except which?

4)Peroxisomes are the site of what? What properties do peroxisomes share with mitochondria?

i need question number nine please

Case Study Section 2 – glycolysis
Based on “Monday at the Metabolic Clinic, Adventures in Glycolysis” by Nancy Boury (National Center for Case Study Teaching in Science)

Background:
You have been chosen for a very competitive undergraduate clinical internship at St. Visintainer’s Hospital and Metabolic Clinic working with Dr. Saccharo, an expert in enzyme deficiency disorders related to glycolysis. Before your first day Dr. Saccharo has asked that you review the reactions of glycolysis and the concept of isoenzymes.

Recall that isoenzymes are groups of enzymes that catalyze the same reaction in that they use the same reactants to product the same products. They may have different genes, tissue expression, developmental timing of expression, and affinities for their substrates. As an example, phosphofructokinase-1 has 3 isoenzyme forms: PFK-L is expressed in the liver and the gene is found on chromosome 21; PFK-M is expressed in the muscle and the gene is found on chromosome 12; and PFK-P is expressed in platelets and the gene is found on chromosome 10.

Question 1:
If a person would be deficient in all 3 forms of PFK-1, this person would .
A. Have lower than normal glycogen stores.
B. Have more mitochondrial activity than normal.
C. Be dead.
D. Produce more energy per glucose molecule because glycolysis would be unregulated.

It is now Monday morning and you are ready to start seeing patients with Dr. Saccharo.

Patient 1:
Ann is a teenager and avid golfer who was referred to the clinic after being refused at the blood drive and tiring easily on the high school golf course during practice. Ann is examined and a blood sample was obtained for analysis of a glycolysis related metabolic panel. The results of her panel is below (levels: + = normal, - = decreased; ++= increased):
Blood Metabolic Panel
Blood Glucose +
Glucose-6-phosphate ++
Fructose-6-phosphate ++
Fructose-1,6,bisphosphate ++
Glyceraldehyde-3-phosphate ++
1,3-bisphosphoglycerate ++
Phosphoenolpyruvate ++
Pyruvate -
ATP -
Red Blood Cell Concentration -
Question 2:
Assuming that Dr. Saccharo is correctly assuming that Ann has a defect in glycolysis, what glycolytic enzyme is the most likely to have the defect based on the blood metabolic profile?
A. Hexokinase
B. Phosphofructokinase
C. Triose Phosphate Isomerase
D. Pyruvate Kinase
E. Aldolase

Patient 2:
Marie is a 32-year-old mother of three complaining of fatigue and muscle cramps with exercise. She had always blamed her intolerance to exercise on her sedentary lifestyle. However, she recently joined a gym and after a week of aerobics classes went to her physician, who ordered a series of blood tests. The blood work came back with abnormal results, leading to her muscle biopsy and referral to the metabolic clinic. Marie’s blood was subjected to the same metabolic panel used before with the following results:
Blood Metabolic Panel
Blood Glucose +
Glucose-6-phosphate ++
Fructose-6-phosphate ++
Fructose-1,6,bisphosphate -
Glyceraldehyde-3-phosphate -
1,3-bisphosphoglycerate -
Pyruvate -
ATP -
Red Blood Cell Concentration -

Marie’s muscle biopsy was also analyzed and showed an excess of glycogen.


Question 3:
What is the most likely enzyme deficiency for Marie?
A. Hexokinase
B. Phosphofructokinase
C. Triose Phosphate Isomerase
D. Pyruvate Kinase
E. Aldolase

Question 4:
If you wanted to test the ability of red blood cells to complete glycolysis what compound would you try to detect?
A. Fructose-6-phosphate
B. Aldolase
C. Pyruvate
D. Phosphofructokinase
E. Dihydroxyacetone phosphate


Question 5:
If you used a sample of Marie’s blood for this, what compound could you add to enable these cells to complete glycolysis?
A. Glucose
B. Fructose-1,6-bisphosphate
C. Insulin
D. Glucagon
E. Fructose-6-phosphate


Patient 3:
Leo is a 25-year-old accountant that is not anemic, has perfect teeth, but has experienced two fainting spells recently. One happened after accidentally drinking a non-diet soda; the other after eating “naturally sweetened” fruit salad. His dentist referred him to Dr. Saccharo’s clinic. Dr. Saccharo suspects Leo may have Hereditary Fructose Intolerance (HFI) and has recommended a closely monitored fructose tolerance test.

Hereditary Fructose Intolerance is a recessive disorder where individuals lack a functional copy of one isoform of the enzyme aldolase, specifically Aldolase B (also known as fructose-1-phosphate aldolase). In the liver, fructose is phosphorylated by Fructokinase to fructose-1-phosphate, then is cleaved by Aldolase B to glyceraldehyde and dihydroxyacetone phosphate. Fructose-1-phosphate is also a known allosteric inhibitor of Glycogen Phosphorylase, the primary enzyme responsible for releasing glucose from glycogen.

Question 6:
As compared to a normal individual that consumes fructose, a person with HFI who ingested fructose would have (select all that apply):
A. High levels of blood fructose
B. High levels of blood fructose-1-phosphate
C. High levels of blood glucose
D. Low levels of blood fructose
E. Low levels of blood glucose


That afternoon Leo undergoes the fructose tolerance test with the following results:

Question 7:
Based on the results of the test, what was the effect of giving Leo the intravenous fructose?
A. A rise in blood glucose levels
B. A decrease in blood glucose levels
C. An increase in glycogen stores
D. A rapid drop in blood pressure


However there were complications: Soon after the test Leo orders and drinks a cup of tea, but after half the cup Leo passes out. Dr. Saccharo orders two sequential blood glucose checks. The first was 50 mg/dl and 15 minutes later Leo’s blood glucose was down to 40 mg/dl. With those results Dr. Saccharo injected something into Leo’s IV and within minutes Leo was back to normal.

Question 8:
From these results does it appear that Leo has HFI?
A. Yes
B. No
C. It is unclear from the results


Question 9:
What did Dr. Saccharo inject into Leo’s IV line?
A. Insulin
B. Glucagon
C. Glucose
D. Fructose
E. Phosphofructokinase

Dr. Saccharo puts Leo on a restricted diet and orders follow-up visits.

Question 10:
Which of the following foods/ingredients would Leo NOT be allowed to eat?
A. Artificial sweeteners
B. High starch foods, such as potatoes
C. Sweet tea made with cane sugar
D. Any dairy products

Dr. Saccharo puts Leo on a restricted diet and orders follow-up visits.

Case Study Section 2 – glycolysis
Based on “Monday at the Metabolic Clinic, Adventures in Glycolysis” by Nancy Boury (National Center for Case Study Teaching in Science)

Background:
You have been chosen for a very competitive undergraduate clinical internship at St. Visintainer’s Hospital and Metabolic Clinic working with Dr. Saccharo, an expert in enzyme deficiency disorders related to glycolysis. Before your first day Dr. Saccharo has asked that you review the reactions of glycolysis and the concept of isoenzymes.

Recall that isoenzymes are groups of enzymes that catalyze the same reaction in that they use the same reactants to product the same products. They may have different genes, tissue expression, developmental timing of expression, and affinities for their substrates. As an example, phosphofructokinase-1 has 3 isoenzyme forms: PFK-L is expressed in the liver and the gene is found on chromosome 21; PFK-M is expressed in the muscle and the gene is found on chromosome 12; and PFK-P is expressed in platelets and the gene is found on chromosome 10.

Question 9:
What did Dr. Saccharo inject into Leo’s IV line?
A. Insulin
B. Glucagon
C. Glucose
D. Fructose
E. Phosphofructokinase