Carbohydrate Metabolism Bcb Test 2

Front 1

Regulation of glycolysis: Inhibitors of phosphofructokinase?

Back 1

ATP, Citrate

Front 2

Regulation of glycolysis: Activators of phosphofructokinase?

Back 2

AMP, ADP, fructose 2,6 bisphosphate

Front 3

Regulation of glycolysis: Hexokinase is inhibited by?

Back 3

glucose 6 phosphate

Front 4

Regulation of glycolysis: Where is hexokinase? glucokinase? Which has higher Km?

Back 4

Hexokinase = muscles, Glucokinase = liver. Glucokinase has higher Km

Front 5

Regulation of glycolysis: Liver isoform of pyruvate kinase is inhibited by?

Back 5

ATP, alanine, and phosphorylation (by PKA)

Front 6

Regulation of glycolysis: Liver isoform of pyruvate kinase is activated by?

Back 6

fructose 1,6 bisphosphate

Front 7

Regulation of glycolysis: Glucose transporter in the muscle is _______ and is activated by ____

Back 7

GLUT4 activated by insulin

Front 8

Regulation of glycolysis: Glucose transporter in the liver is _______ and it _______

Back 8

GLUT2 in liver equilibrates

Front 9

Regulation of glycolysis: Classical inhibitor of glycolysis is? at which step?

Back 9

Classical inhibitor—iodoacetate (glyceraldehyde-3-P dehydrogenase)

Front 10

Pyruvate dehydrogenase: Overall reaction?

Back 10

pyruvate + NAD + CoA -> Acetyl-CoA + CO2 + NADH

Front 11

Pyruvate dehydrogenase: Bound cofactor of pyruvate dehydrogenase?

Back 11

TPP = thiamine pyrophosphate

Front 12

Pyruvate dehydrogenase: Bound cofactor of dihydrolipolyl transacetylase?

Back 12

Lipoic Acid linked to lysine side chain

Front 13

Pyruvate dehydrogenase: Bound cofactor of dihydrolipolyl dehydrogenase?

Back 13

FAD

Front 14

Regulation of pyruvate dehydrogenase: inhibited by?

Back 14

high acetyl-CoA/CoA and NADH/NAD ratios and by a specific protein kinase

Front 15

Regulation of pyruvate dehydrogenase: The kinase that inhibits PDH is activated by?

Back 15

high acetyl-CoA/CoA, ATP/ADP, NADH/NAD ratios

Front 16

Regulation of pyruvate dehydrogenase: the phosphatase that can activate PDH is activated by?

Back 16

pyruvate and insulin

Front 17

ETC: Complex 1?

Back 17

NADH-Q Reductase

Front 18

ETC: Complex 2?

Back 18

Succinate dehydrogenase (with FAD)

Front 19

ETC: ACD?

Back 19

acetyl CoA dehydrogenase (next to complex 2)

Front 20

ETC: Complex 3?

Back 20

QH2-cytochrome c reductase

Front 21

ETC: Complex 4?

Back 21

cytochrome oxidase

Front 22

ETC: Complex 5?

Back 22

ATP synthase

Front 23

ETC Sequence?

Back 23

NADH > Complex1 (ATP) > CoQ (4 inputs) > Complex 3 cyto B (ATP) > Complex 3 cyto c1 > cyto c > complex 4 (ATP) > O2

Front 24

Oxidative Phosphorylation P/O ratios?

Back 24

3 ATP per NADH & 2 ATP per FADH2 (classical numbers)

Front 25

ETC: Site 1 inhibitor? complex 1?

Back 25

Rotenone, Amytal

Front 26

ETC: Site 2 inhibitor? (complex 3?)

Back 26

Antimycin A

Front 27

ETC: Site 3 inhibitor? (complex 4?)

Back 27

HCN (cyanide), CO (carbon monoxide, H2S, N3- (azide)

Front 28

ETC: F0 =? F1=?

Back 28

F0 = pore, F1 = ATPase

Front 29

Oligomycin acts by blocking the _____?

Back 29

F0 pore

Front 30

Notable uncouplers?

Back 30

ionophores valinomycin and K+ -> destroys membrane potential

DNP

Front 31

Adenine nucleotide translocase inhibitors?

Back 31

atractyloside and bongkrekic acid

Front 32

Regulation of gluconeogenesis: Pyruvate carboxylase has required activator?

Back 32

acetyl-CoA

Front 33

Regulation of gluconeogenesis: Fructose 1,6-bisphosphatase is inhibited by?

Back 33

AMP & fructose 2,6-bisphosphate

Front 34

Regulation of gluconeogenesis: Liver pyruvate kinase must be ______ for gluconeogensis to proceed?

Back 34

inhibited

Front 35

Regulation of gluconeogenesis: Liver pyruvate kinase is activated by?

Back 35

fructose 16 Bisphosphate

Front 36

Regulation of gluconeogenesis: Liver pyruvate kinase is inhibited by?

Back 36

ATP, alanine, and by phosphorylation (by PKA)

Front 37

Glycogenolysis: Glycogen phosphorylase cleaves ____-linkages only up to ___ residues from branch point into ____?

Back 37

cleaves 1,4-linkages only, at least 4 residues from branch point into G1P

Front 38

Glycogenolysis: __________ cleaves 1,4-linkages only, at least 4 residues from branch point?

Back 38

Glycogen phosphorylase

Front 39

Glycogenolysis: __________ converts glucose-1-P to glucose-6-P? G6P can then?

Back 39

Phosphoglucomutase converts glucose-1-P to glucose-6-P. G6P can then go to glucose, glycolysis, or Pentose phosphate pathway

Front 40

Glycogenolysis: ______ moves all but the last glucose residue in a branch?

Back 40

Transferase

Front 41

Glycogenolysis: ________hydrolyzes the 1,6 linkage of the last residue of a branch

Back 41

alpha-1,6-glucosidase

Front 42

Glycogen synthesis: Glucose-1-P + UTP -> UDP-glucose + pyrophosphate. Enzyme=?

Back 42

UDP-glucose pyrophosphorylase

Front 43

Glycogen synthesis: Glycogen(n) + UDP-glucose -> glycogen(n+1) + UDP (1,4-linkages). Enzyme?

Back 43

Glycogen synthase

Front 44

Glycogen regulation: The unphosporylated form of glycogen synthase is known as? active or inactive?

Back 44

unphosphorylated = glycogen synthase A = active

Front 45

Glycogen regulation: The phosporylated form of glycogen synthase is known as? active or inactive?

Back 45

phosphorylated = glycogen synthase B = inactive

Front 46

Glycogen regulation: activation of PKA by cAMP ______ glycogen synthesis while it _______ glycogen breakdown

Back 46

inhibits glycogen synthesis promotes glycogen breakdown

Front 47

Glycogen regulation: Phosphorylase b is activated by? inhibited by?

Back 47

activated/dependent on AMP and inhibited by ATP, glucose-6-P

Front 48

Glycogen regulation: Phosphorylase b kinase (unphosphorylated form) can be activated by ___ and ____.

Back 48

PKA and Ca2+

Front 49

Cyclic AMP is removed by conversion to ____by ______

Back 49

Cyclic AMP is removed by conversion to AMP by phosphodiesterase

Front 50

Phosphodiesterases (PDEs) are inhibited by?

Back 50

methyl xanthines such as caffeine.

Front 51

Insulin activates glycogen synthase via inhibition of?

Back 51

GSK3 (glycogen synthase kinase 3)

Front 52

In muscle, insulin also stimulates glycogen synthesis via activation of?

Back 52

glucose transport (Glut4)

Front 53

The first two enzymes of the urea cycle, ________ and _______________ are located in the ___________, whereas the other enzymes are ______________.

Back 53

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.

Front 54

Carbamoyl phosphate synthetase 1 is controlled by a specific activator = ?

Back 54

acetyl-glutamate

Front 55

Carbamoyl phosphate synthetase 1 is also controlled by the concentration of ?

Back 55

concentration of the substrate ammonia

Front 56

glutamate dehydrogenase reaction

Back 56

Glutamate + NAD(P) -> alpha-ketoglutarate + NADPH + NH3

Front 57

Glutamine is the main carrier for excess nitrogen produced where?

Back 57

in peripheral tissues other than muscle

Front 58

Other amino acids that result in free ammonia?

Back 58

serine, threonine, and histidine

Front 59

PKU is a genetic disease of amino acid (AA) metabolism specifically involving the metabolism of ?

Back 59

tyrosine

Front 60

tyr is synthesized from______ via ______

Back 60

Tyrosine is synthesized from phenylalanine via phenylalanine hydroxylase (PH)

Front 61

Result of Phenylalanine hydroxylase mutation?

Back 61

Tryosine become an essential amino acid and Phe accumulates

Front 62

What happens after Phe accumulates?

Back 62

Phe degradation is increased

Front 63

Increased Phe degredation results in increased

Back 63

phenylpyruvic acid

Front 64

Further metabolites of phenylpyruvic acid?

Back 64

phenyllactate, phenylacetate and phenylacetyl glutamine

Front 65

In some individuals, ___________ is (genetically) limiting and also results in the symptoms of PKU.

Back 65

BH2 reductase

know the conversion step of Phe -> tyr

Front 66

maple syrup urine disease?

Back 66

mutation of brached chain alpha-keto acid dehydrogenase

(leucine, valine, isoleucine)

Front 67

significant finding in alcaptonuria?

Back 67

black urine