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PHAR 412

Carbohydrates, Lipids, Proteins, and Amino Acids

QuestionAnswer
In which tissues does galactose metabolism occur? Liver, erythrocytes, fibroblasts
What enzyme is defective in hereditary fructose intolerance? Aldolase B
What enzyme is deficient in essential fructosuria? Fructokinase
For what purpose is fructose made in humans? Fuel for spermatazoa
In which tissue is fructose made for spermatozoa fuel? Seminal vesicles
What is the cause of classical galactosemia? Galactose 1-phosphate uridyl transferase deficiency
What is the cause of non-classical galactosemia? What are the symptoms? Galactokinase deficiency; cataracts, no brain damage
What type of glycosidic bond does lactose have? B-1,4
What type of glycosidic bond does sucrose have? a, B-1,2
What enzyme is responsible for digesting Cellobiose (glucose B-1,4 glucose)? None
What enzyme is responsible for digesting Maltose (glucose a-1,4 glucose)? Glucoamylase / sucrase-isomaltase
What enzyme is responsible for digesting Isomaltose (glucose a-1,6 glucose)? Sucrase-Isomaltase
What enzyme is responsible for digesting Lactose (galactose B-1,4 glucose)? Lactase
What enzyme is responsible for digesting sucrose (glucose a, B-1,2 fructose)? Sucrase
What enzyme is responsible for digesting trehalose (glucose a, a-1,1 glucose)? Trehalase
What disaccharide cannot be digested by the sucrose-isomaltase complex? glucose a,a-1,1 glucose
GLUT-1 Red blood cells, blood brain barrier
GLUT-2 Liver, Pancreatic B-cells, contraluminal membrane of intestinal epithelial cells (accepts all 3 monosaccharides)
GLUT-3 Brain
GLUT-4 Adipose tissue, skeletal muscle, heart muscle (insulin sensitive)
GLUT-5 Intestinal epithelial cells, sperm (fructose transporter)
SGLT-1 Intestinal epithelial cells (Na dependent)
SGLT-2 Kidney (Na dependent)
Glucose uptake by muscle is stimulated by what? AMP
AMP-stimulated protein kinase translocation of GLUT-4 is generated in what reaction? Adenylate kinase reaction
The liver is insulin dependent/independent? Independent
True/False: Under low glucose conditions glucokinase is bound to GKRP in the hepatocyte nucleus. True
True/False: Under high glucose conditions glucokinase is bound to GKRP in the hepatocyte nucleus. False, it is released from GKRP, travels to cytoplasm and phosphorylates glucose.
True/False: Hexokinase is found in all tissues and is inhibited by glucose 6-phosphate. True
True/False: In the absence of a glycogen fragment, a specific protein, glycogenin, can serve as an acceptor of glucose residues. True
True/False: Glycogenin transfers a few molecules of glucose from UDP-glucose to form a short chain to be further elongated by glycogen synthase. True
The enzyme responsible for cleaving a block of 5-8 residues and reattaching them at a more interior site is Glucosyl transferase
What does phosphorylase do to glucose residues? Removes glucose residues from the nonreducing ends to form glucose 1-P.
What does glycogen synthase do to a glycogen molecule? It transfers glucose from UDPG to a nonreducing end of a pre-existing glycogen molecule.
What does phosphoprotein phosphatase-1 do when bound to its inhibitor? Once the inhibitor is activated by phosphorylation, it will bind to the phosphatase and inactivate it, removing its inhibition on active phosphorylase and inactive synthase.
What three things does protein kinase A do? Activates phosphorylase, inactivates synthase, and activates phosphatase inhibitor, all by phosphorylation.
What are the key differences between epinephrine and glucagon? Epinephrine acts in liver and muscle, glucagon acts just in liver. Epinephrine comes from adrenal medulla and glucagon comes from a-cells of pancreas.
True/False: Glucagon binds to the glucagon receptor in the liver and activates adneylate cyclase, via G-proteins. cAMP binds to protein kinase A. True
True/False: Protein kinase A activates phosphorylase kinase by phosphorylation, which in turn, phosphorylates glycogen phosphorylase b, thereby converting it to the active phosphorylase a. True
What are three kinases that phosphorylate glycogen synthase? Protein kinase A, phosphorylase kinase, glycogen synthase kinase-3 (GSK-3)
What enzyme is activated by insulin and decreases cAMP? Phosphodiesterase
What membrane bound enzyme is activated when epinephrine binds to a-receptors in the liver that involve G-proteins as the activator? Phosholipase C (PLC)
What two products are formed when PLC hydrolyzes phosphatidylinositol bisphosphate (PIP)? Diacylglycerol (DAG) and inositol triphosphate (IP3)
What does IP3 stimulate the release of from the endoplasmic reticulum? Calcium
Protein kinase C is activated by what two things? Calcium and DAG
The calcium-calmodulin complex activates what two enzymes? Calmodulin-dependent protein kinase & Phosphorylase kinase
What three enzymes all phosphorylate glycogen synthase when epinephrine is bound to a-agonist receptors? Protein kinase C, Calmodulin-dependent protein kinase, and Phosphorylase kinase
True/False: Insulin inactivates glycogen synthase kinase-3. True
AMP formed from ATP during muscular contraction binds to and activates what enzyme? Glycogen phosphorylase b (active without phosphorylation)
Where is calcium released from after nerve impulses initiate contraction? Sarcoplasmic reticulum
Once calcium is released into the cytosol of a muscle cell it binds to calmodulin, making a calcium-calmodulin complex that binds to and activates what enzyme? Phosphorylase kinase (active without phosphorylation)
True/False: The cascade caused by epinephrine in muscle is the same as in the liver. True
What is the defective enzyme in Von Gierke's Disease? Glucose 6-phosphatase; causes massive enlargement of liver, hypoglycemia, ketosis, hyperuricemia, hyperlipemia
What is the defective enzyme in Pompe's Disease? a-1,4-glucosidase; causes cardiorespiratory failure and causes death before age 2.
What is the defective enzyme in Cori's Disease? amylo-1,6-glucosidase (debranching enzyme); causes massive enlargement of the liver, hypoglycemia, ketosis, hyperuricemia, hyperlipemia (milder course); muscle and liver.
What is the defective enzyme in Andersen's Disease? Branching enzyme; causes progressive cirrhosis of the liver and causes death before age 2.
What is the defective enzyme in McArdle's Disease? Phosphorylase; causes limited ability to perform exercise because of cramps.
What is the defective enzyme in Hers' Disease? Phosphorylase; causes massive enlargement of the liver, hypoglycemia, ketosis, hyperuricemia, hyperlipemia.
What is the defective enzyme in VIII? Phosphorylase kinase; causes mild liver enlargement and mild hypoglycemia.
Created by: sdeck86