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Carbohydrates IV
E. Pennington
This work is produced by The Connexions Project and licensed under the Creative Commons Attribution License †
Abstract Final exam for all Carbohydrate lectures.
- A 21-year old male joins the military. He is required to do exercise during basic training. He experiences pain, cramps and fatigue after 20 minutes of strenuous physical activity and his urine turns burgundy- colored. Lab results showed after exercise: high serum creatine levels and myoglobinuria. An ischemic forearm exercise test showed: low lactate and high ammonia levels in blood. A muscle biopsy reveals:
Glycogen 1.85 mmol/min/g (Normal values: 0.1 - 1.5 mmol/min/g) Phosphorylase A 0.09 mmol/min/g (Normal values: 12 mmol/min/g) What condition is this patient suering from?
A. McArdles' disease type V B. Andersons' disease type IV C. von Gierke's disease type I D. Hers' disease type VI E. Cori's disease type III
- Which of the following hepatic enzymes connects glycogenolysis to the last step of gluconeogenesis?
A. Glycogenin B. Branching enzyme C. Phosphoglucomutase D. Glucose-6-phosphatase E. Glycogen phosphorylase
- Which of the following proteins is directly involved in glycogenolysis in the liver and muscle cells?
A. Glycogenin B. Phosphoglucomutase C. Branching enzyme D. Glycogen synthase E. Insulin
- Which of the following statements about bers is INCORRECT?
∗Version 1.1: Dec 23, 2011 8:59 am US/Central †http://creativecommons.org/licenses/by/3.0/
A. Soluble bers are fermented by normal intestinal bacteria. B. Soluble bers slow the absorption of glucose into circulation. C. Soluble bers can prevent heart disease by reducing cholesterol in circulation. D. Insoluble bers increase the regularity of bowel movements and prevent constipation. E. The products of insoluble bers are gases, short fatty acids, lactate and vitamin K.
- What is the main purpose of glucuronidation reactions?
A. Stimulate abnormal cell proliferation in the tissues. B. Activate other enzymes to release non-polar molecules. C. Get rid of drugs faster from the body without a therapeutic eect. D. Add two glucose molecules to non-polar molecules to make them polar. E. Clear insoluble molecules by excreting them via the urine and feces.
- Which of the following enzymes is responsible for the development of cataracts in galactosemic patients?
A. Galactose-1-phosphate uridyltransferase B. UDP-galactose-4-epimerase C. Sorbitol dehydrogenase D. Galactokinase E. Aldose reductase
- Which of the following enzymes actively breaks down complex carbohydrates in both the mouth and the lumen of the intestines?
A. α-amylase B. Sucrase C. Isomaltase D. ÿ-glucoamylase E. ÿ-glycosidase
- Which of the following is the best screening test to detect GLUT1 mutations?
A. CSF glucose levels B. Serum glucose levels C. SLC2A1 genetic testing D. CSF per Blood glucose ratios E. F 18 positron emission tomography
- Which of the following proteins is found in high concentration in colostrum?
A. α-lactalbumin B. Immunoglobulin A C. Lysozyme D. Insulin E. Casein
- A 7-month old baby is being introduced to solid foods. Mother notices her baby gets fussy, and suers from chronic bloating and diarrhea every time he eats bananas, apples or pears. A stool reducing substances test is positive. Which of the following proteins might be defective in this patient?
A. Sucrase B. α-amylase C. ÿ-glucoamylase
- A 1-month old male is failing to thrive. Lab results showed hypoglycemia accompanied by ketosis during fasting and low blood levels of lactate and alanine. Although feeding relieves the symptoms, it results in post-prandial hyperglycemia and hyperlacticacidemia. A liver biopsy revealed decreased glycogen stores in the liver. There is not enlargement of the liver as a result of storage of excessive or abnormal glycogen. Which of the following enzymes is responsible for the lack of enlongated terminal ends (A-chains) in the stored glycogen of this patient?
A. Glycogenin B. Branching enzyme C. Debranching enzyme D. Glycogen synthase E. Glycogen phosphorylase
- Which of the following is a benign disorder that can have normal blood lab values?
A. Fructosuria B. Fructose Intolerance C. Classical galactosemia D. UDP-galactose-4-epimerase
- Which of the following hepatic metabolic pathways WOULD NOT be aected by a de cient aldolase B?
A. Glycolysis B. Glycogenesis C. Glycogenolysis D. Gluconeogenesis E. Fructose metabolism
- Which of the following hormones controls the synthesis and activity of glycogen synthase during the fed state?
A. Renin B. Cortisol C. Insulin D. Glucagon E. Epinephrine
- Which of the following hormones stimulates the synthesis of the enzymes for lactogenesis?
A. Aldosterone B. Epinephrine C. Prolactin D. Insulin E. Glucagon
- Which of the following protein transporters has the capability to allow glucose, galactose and fructose into the hepatic portal vein circulation?
A. GLUT B. GLUT C. GLUT D. GLUT
E. GLUT
Questions 23 to 25 will use the same clinical information to be answered.
- A newborn is initially breastfed. He develops the following symptoms when he feeds: vomiting, diarrhea, convulsions, irritability, lethargy and refuses to ingest breast milk. He begins to lose weight. Which of the following conditions should be ruled out in this patient?
- Fructose intolerance
- Classical galactosemia
- Fructose malabsorption
- UDP-galactose-4-epimerase de ciency
- Blood lab tests of this newborn (Question 23 above) showed: hypoglycemia, aminoaciduria, hepatomegaly and impaired liver function. A screen for reducing substances in the urine was positive. Carbohydrate chromatography showed high levels of galactose and galactitol in urine. Which of the following enzymes is defective in this infant?
A. Aldolase B B. Fructokinase C. Galactokinase D. Galactosyltransferase E. Galactose-1-phosphate uridyltransferase
- Which of the following milk products can be used to feed this newborn patient? (Patient in questions 23 & 24 above)
A. Colostrum B. Cow's milk C. Goat's milk D. Soy formula E. Mother's breast milk
