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67 Cards in this Set
- Front
- Back
Regulation of glycolysis: Inhibitors of phosphofructokinase?
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ATP, Citrate
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Regulation of glycolysis: Activators of phosphofructokinase?
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AMP, ADP, fructose 2,6 bisphosphate
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Regulation of glycolysis: Hexokinase is inhibited by?
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glucose 6 phosphate
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Regulation of glycolysis: Where is hexokinase? glucokinase? Which has higher Km?
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Hexokinase = muscles, Glucokinase = liver. Glucokinase has higher Km
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Regulation of glycolysis: Liver isoform of pyruvate kinase is inhibited by?
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ATP, alanine, and phosphorylation (by PKA)
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Regulation of glycolysis: Liver isoform of pyruvate kinase is activated by?
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fructose 1,6 bisphosphate
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Regulation of glycolysis: Glucose transporter in the muscle is _______ and is activated by ____
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GLUT4 activated by insulin
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Regulation of glycolysis: Glucose transporter in the liver is _______ and it _______
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GLUT2 in liver equilibrates
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Regulation of glycolysis: Classical inhibitor of glycolysis is? at which step?
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Classical inhibitor—iodoacetate (glyceraldehyde-3-P dehydrogenase)
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Pyruvate dehydrogenase: Overall reaction?
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pyruvate + NAD + CoA -> Acetyl-CoA + CO2 + NADH
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Pyruvate dehydrogenase: Bound cofactor of pyruvate dehydrogenase?
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TPP = thiamine pyrophosphate
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Pyruvate dehydrogenase: Bound cofactor of dihydrolipolyl transacetylase?
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Lipoic Acid linked to lysine side chain
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Pyruvate dehydrogenase: Bound cofactor of dihydrolipolyl dehydrogenase?
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FAD
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Regulation of pyruvate dehydrogenase: inhibited by?
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high acetyl-CoA/CoA and NADH/NAD ratios and by a specific protein kinase
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Regulation of pyruvate dehydrogenase: The kinase that inhibits PDH is activated by?
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high acetyl-CoA/CoA, ATP/ADP, NADH/NAD ratios
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Regulation of pyruvate dehydrogenase: the phosphatase that can activate PDH is activated by?
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pyruvate and insulin
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ETC: Complex 1?
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NADH-Q Reductase
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ETC: Complex 2?
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Succinate dehydrogenase (with FAD)
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ETC: ACD?
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acetyl CoA dehydrogenase (next to complex 2)
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ETC: Complex 3?
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QH2-cytochrome c reductase
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ETC: Complex 4?
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cytochrome oxidase
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ETC: Complex 5?
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ATP synthase
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ETC Sequence?
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NADH > Complex1 (ATP) > CoQ (4 inputs) > Complex 3 cyto B (ATP) > Complex 3 cyto c1 > cyto c > complex 4 (ATP) > O2
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Oxidative Phosphorylation P/O ratios?
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3 ATP per NADH & 2 ATP per FADH2 (classical numbers)
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ETC: Site 1 inhibitor? complex 1?
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Rotenone, Amytal
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ETC: Site 2 inhibitor? (complex 3?)
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Antimycin A
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ETC: Site 3 inhibitor? (complex 4?)
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HCN (cyanide), CO (carbon monoxide, H2S, N3- (azide)
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ETC: F0 =? F1=?
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F0 = pore, F1 = ATPase
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Oligomycin acts by blocking the _____?
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F0 pore
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Notable uncouplers?
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ionophores valinomycin and K+ -> destroys membrane potential
DNP |
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Adenine nucleotide translocase inhibitors?
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atractyloside and bongkrekic acid
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Regulation of gluconeogenesis: Pyruvate carboxylase has required activator?
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acetyl-CoA
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Regulation of gluconeogenesis: Fructose 1,6-bisphosphatase is inhibited by?
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AMP & fructose 2,6-bisphosphate
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Regulation of gluconeogenesis: Liver pyruvate kinase must be ______ for gluconeogensis to proceed?
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inhibited
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Regulation of gluconeogenesis: Liver pyruvate kinase is activated by?
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fructose 16 Bisphosphate
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Regulation of gluconeogenesis: Liver pyruvate kinase is inhibited by?
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ATP, alanine, and by phosphorylation (by PKA)
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Glycogenolysis: Glycogen phosphorylase cleaves ____-linkages only up to ___ residues from branch point into ____?
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cleaves 1,4-linkages only, at least 4 residues from branch point into G1P
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Glycogenolysis: __________ cleaves 1,4-linkages only, at least 4 residues from branch point?
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Glycogen phosphorylase
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Glycogenolysis: __________ converts glucose-1-P to glucose-6-P? G6P can then?
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Phosphoglucomutase converts glucose-1-P to glucose-6-P. G6P can then go to glucose, glycolysis, or Pentose phosphate pathway
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Glycogenolysis: ______ moves all but the last glucose residue in a branch?
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Transferase
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Glycogenolysis: ________hydrolyzes the 1,6 linkage of the last residue of a branch
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alpha-1,6-glucosidase
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Glycogen synthesis: Glucose-1-P + UTP -> UDP-glucose + pyrophosphate. Enzyme=?
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UDP-glucose pyrophosphorylase
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Glycogen synthesis: Glycogen(n) + UDP-glucose -> glycogen(n+1) + UDP (1,4-linkages). Enzyme?
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Glycogen synthase
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Glycogen regulation: The unphosporylated form of glycogen synthase is known as? active or inactive?
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unphosphorylated = glycogen synthase A = active
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Glycogen regulation: The phosporylated form of glycogen synthase is known as? active or inactive?
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phosphorylated = glycogen synthase B = inactive
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Glycogen regulation: activation of PKA by cAMP ______ glycogen synthesis while it _______ glycogen breakdown
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inhibits glycogen synthesis promotes glycogen breakdown
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Glycogen regulation: Phosphorylase b is activated by? inhibited by?
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activated/dependent on AMP and inhibited by ATP, glucose-6-P
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Glycogen regulation: Phosphorylase b kinase (unphosphorylated form) can be activated by ___ and ____.
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PKA and Ca2+
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Cyclic AMP is removed by conversion to ____by ______
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Cyclic AMP is removed by conversion to AMP by phosphodiesterase
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Phosphodiesterases (PDEs) are inhibited by?
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methyl xanthines such as caffeine.
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Insulin activates glycogen synthase via inhibition of?
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GSK3 (glycogen synthase kinase 3)
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In muscle, insulin also stimulates glycogen synthesis via activation of?
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glucose transport (Glut4)
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The first two enzymes of the urea cycle, ________ and _______________ are located in the ___________, whereas the other enzymes are ______________.
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The first two enzymes of the urea cycle, carbamoyl phosphate synthetase 1 and ornithine transcarbamoylase are located in the mitochondrial matrix, whereas the other enzymes are cytosolic.
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Carbamoyl phosphate synthetase 1 is controlled by a specific activator = ?
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acetyl-glutamate
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Carbamoyl phosphate synthetase 1 is also controlled by the concentration of ?
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concentration of the substrate ammonia
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glutamate dehydrogenase reaction
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Glutamate + NAD(P) -> alpha-ketoglutarate + NADPH + NH3
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Glutamine is the main carrier for excess nitrogen produced where?
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in peripheral tissues other than muscle
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Other amino acids that result in free ammonia?
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serine, threonine, and histidine
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PKU is a genetic disease of amino acid (AA) metabolism specifically involving the metabolism of ?
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tyrosine
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tyr is synthesized from______ via ______
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Tyrosine is synthesized from phenylalanine via phenylalanine hydroxylase (PH)
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Result of Phenylalanine hydroxylase mutation?
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Tryosine become an essential amino acid and Phe accumulates
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What happens after Phe accumulates?
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Phe degradation is increased
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Increased Phe degredation results in increased
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phenylpyruvic acid
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Further metabolites of phenylpyruvic acid?
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phenyllactate, phenylacetate and phenylacetyl glutamine
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In some individuals, ___________ is (genetically) limiting and also results in the symptoms of PKU.
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BH2 reductase
know the conversion step of Phe -> tyr |
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maple syrup urine disease?
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mutation of brached chain alpha-keto acid dehydrogenase
(leucine, valine, isoleucine) |
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significant finding in alcaptonuria?
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black urine
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