Quiz 1 LOs Flashcards
What is the rate limiting enzyme of glycolysis?
What 2 molecules activate it?
What 2 molecules inhibit it?
PFK-1
ACTIVATE: AMP, F-2,6-BP
INHIBIT: ATP, citrate
Which of the following glucose transporters is located in the liver with the lowest affinity, and which is regulated by insulin?
GLUT1
GLUT2
GLUT3
GLUT4
GLUT2 is in liver with lowest affinity
GLUT4 is regulated by insulin
Pyruvate kinase is a major regulated step in glycolysis. What 2 molecules stimulate activation, and what 3 molecules inhibit its activity?
Note: Pyruvate kinase works and you’re “in”, but when it’s off “GAA”
ACTIVATE: Insulin, F-1,6-BP
INHIBIT: Alanine, ATP, Glucagon
Note: Pyruvate kinase works and you’re “in”, but when it’s off “GAA”
Which of the two hormones is associated with dephosphorylation?
A. Glucagon
B. Insulin
Insulin
Taurus’ are Pretty Fucking Kind, but Tarui disease is not. PTs present with exercise induced cramps, hemolytic anemia, high bilirubin and jaundice. Teuri disease is caused by a deficiency in what major enzyme of glycolysis?
PFK-1
T/F: It seems like insulin and phosphatases is always vs. glucagon and cAMP/PKA
True
Pyruvate can be converted to alanine through what two metabolic processes?
Gluconeogenesis
Urea cycle
About 95% of glycolysis related anemias are associated with defects in what enzyme?
Pyruvate kinase
What are two major clinical markers for hemolytic anemia?
A. Lactate dehydrogenase, conjugated bilirubin
B. Pyruvate kinase, PFK-1
C. Lactate dehydrogenase, unconjugated bilirubin
D. None of the above
Lactate dehydrogenase, unconjugated bilirubin
Fanconi-Bickel syndrome is a “TWO-NAME SYNDROME”, with a lot of words to TRANSPORT. Presentation includes hepatosplenomegaly, failure to thrive, and resistant rickets. What deficiency is causes this two-name syndrome?
GLUT2 defect that prevents transport of glucose, galactose, and fructose to be transported
Which of the following, is an activator for all of the major regulatory steps of gluconeogenesis that include the following enzymes: Pyruvate carboxylase, PEP carboxykinase, F-1,6-Biphosphatase, and Glucose-6-phosphatase?
A. AMP
B. Cortisol
C. Citrate
D. ADP
Cortisol
What is the rate limiting enzyme of gluconeogenesis?
What are the 2 aCCtivators?
What are its 2 inhibitors?
Fructose-1,6-biphosphatase
ACTIVATORS: Citrate, Cortisol
INHIBITORS: AMP, F2,6BP
What are the only 3 activators of PEP carboxykinase that stimulate its activity in gluconeogenesis?
Cortisol
Glucagon
Thyroxine
What are the 2 activators of pyruvate carboxylase and its only ADept inhibitor as it acts in gluconeogenesis?
ACTIVATOR: Cortisol, Acetyl CoA
INHIBITOR: ADP
NOTE: pyruvate carboxylase has an ADept inhibitor
If F2,6BP activates PFK-1 in glycolysis then it makes sense that F2,6BP inhibits what enzyme in gluconeogenesis?
Fructose-1,6-biphosphatase
Which of the following enzymes is the only one to be found in the mitochondria and utilizes biotin (B7)?
A. PEP carboxykinase
B. Fructose-1,6-biphosphatase
C. Glucose-6-phosphatase
D. Pyruvate carboxylase
Pyruvate carboxylase
Tarui disease is caused by a deficiency in PFK-1. What other disease is similar to Tarui disease in gluconeogenesis?
Fructose-1,6-biphosphatase deficiency and Tarui disease present similaraly
Lactic acidosis, hypoglycemia
Explain how glucose is made into fructose in cells that have sorbitol dehydrogenase in the Polyl pathway. Include intermediates and enzymes used.
Glucose + Aldose reductase –> sorbitol + sorbitol dehydrogenase —> fructose
Cells lacking sorbitol dehydrogenase: Kidney, retina, schwann cells
Fructose w/ _________ makes F6P, and enters in glycolysis. While fructose with _______ makes F1P, skipping major regulatory step and entering glycolysis at intermediate Glyceraldehyde 3 phosphate
Hexokinase
Fructokinase
In fructose metabolism, what two enzymes bypass the major regulatory step of glycolysis?
Fructokinase
Triose Kinase
Galactosemia has non-classical and classical versions depending on the deficinent enzyme. What is the deficient enzyme in either version that leads to the buildup of galactose or galactitol causing cataracts?
Classical: GALT/glacatose 1P uridyltransferase
Non-classical: Glactokinase
T/F: Galactose is one step away from Galactitol via aldolase reductase
True
What is the rate limiting enzyme of the PPP?
Glucose-6-phosphate dehydrogenase
Which of the following is the likely result with GSD 0? Bonus: what enzyme is deficient?
A. Long chains with few branches
B. Short chains with many branches
C. Free glucose unable to be converted to glycogen
D. None of the above
Free glucose unable to be converted to glycogen
Enzyme: Glycogen synthase (rate limiting enzyme)
Which of the following is the likely result with Andersen disease (GSD IV)? What enzyme is deficient
A. Long chains with few branches
B. High number of short branches
C. Free glucose unable to be converted to glycogen
D. None of the above
Long chains with few branches
Enzyme: Glucosyl (4:6) transferase
What is the rate limiting enzyme of glycogenolysis and glycogenesis?
Glycogenesis: Glycogen synthase
Glycogenolysis: Glycogen phosphorylase
Which of the following is the likely result with Cori disease (GSD III)? What is the deficient enzyme?
A. Long chains with few branches
B. High number of short branches
C. Free glucose unable to be converted to glycogen
D. None of the above
High number of short branches
a-1,6-glucosidase
In terms of phosphorylation, which form of glycogen synthase is active and which form of glycogen phosphorylase is active?
DEphosphorylated glycogen synthase is active
Phosphorylated glycogen phosphorylase is active
T/F: Glucagon does not act on the muscle
True
ATP citrate lyase is the enzyme responsible for converting citrate back to acetyl CoA and OAA once out in the cytosol in FA synthesis. What are citrate lyase’s 2 activators, and what are the 2 inhibitors?
ACTIVATORS: Glucose, Insulin
INHIBITORS: PUFA, leptin
T/F: Oxaloacetate is unable to enter and exit mitochondria directly
True
In fatty acid synthesis, which of the following acts as an inhibitor in every major phase?
A. PUFA
B. Leptin
C. Insulin
D. Glucagon
PUFA
T/F: Carboxylase enzymes generally utilize biotin as a cofactor
True
What is the rate limiting step and enzyme in fatty acid synthesis?
Acetyl CoA –> Malonyl CoA
Acetyl-CoA carboxylase
The presence of malonyl coA inhibits the function of what rate limiting enzyme in fatty acid degredation, in order to prevent them from occuring at the same time?
Carnitine acyl transferase
Phase III of fatty acid synthesis is chain formation where 2carbon units are added to the fatty acyl chain in 7 reactions to form palmitate. What is the name of the enzyme complex that carries this out?
Fatty Acid Synthase Complex
Fatty acid synthase is a complex of 2 identical dimers made up of 7 enzymes and what additional protein with a flexible arm?
Acyl Carrier protein
What 2 hormones/hormone types stimulate the activity of Fatty Acid Synthase Complex?
Insulin
Glucocorticoid hormones
Because the brain requires longer chain fatty acids (C18-24), it is necessary for palmitate to be lengthened. This addition can only happen in 2 different locations, and each use their own carbon donor plus NADPH for reducing power. What are the 2 locations where this occurs, and what are their respective carbon donors?
Smooth ER uses Malonyl CoA
Mitochondria uses Acetyl CoA
What are the 4 human desaturases and where do they each present a new double bond?
delta4: 4-5
delta5: 5-6
delta6: 6-7
delta9: 9-10
TAGs are made in through de novo synthesis in all of the following locations except for which, which is where dietary TAGs are processed?
A. Hepatocytes
B. Adipocytes
C. Intestinal cells
D. None of the above
Intestinal cells
What is the name of the lipoprotein that contaiins apolipoproteins and other lipids synthesized from the small intestines? These lipoproteins later enter the lymphatic system through the thoracic duct.
A. Chylomacroons
B. VLDLs
C. LDLs
D. Chylomicrons
Chylomicrons
In the de novo synthesis of TAGs made in the liver glucose and glycerol combine to form glycerol-3-phosphate. This glycerol-3-phosphate is used as the backbone and combined with FFAs to form TAGs. These TAGs are packaged into lipoproteins that are called:
A. Chylomacroons
B. VLDLs
C. LDLs
D. Chylomicrons
VLDLs