Xxx - Bchm Iv - Pathways

Front 1

ATP METABOLISM

- Aerobic pathway shuttle pathways
- how much ATP is made for each?

Back 1

Malate-Aspartate shuttle (32 ATP)

Glycerol-3-Phosphate shuttle (30 ATP)

Front 2

ATP METABOLISM

- Malate-Aspartate shuttle utilized where?

- Glycerol-3-Phosphate shuttle utilized where?

Back 2

- Heart
- Liver

- Muscle

Front 3

ATP METABOLISM

- Anaerobic metabolism of glucose produces how many ATPs?

Back 3

- 2 ATP

Front 4

NADPH

- is a product of what pathway?

Back 4

- HMP shunt

Front 5

NADPH

- used in what/where? x5

Back 5

(NADPH GRAPE)

- Glutathione Reductase
- Respiratory Burst
- Anabolic processes
- P450
- Electron ACCEPTOR (universal)

Front 6

NAD+ is used in what processes?

Back 6

- Catabolic

Front 7

NADPH is used in what processes?

Back 7

- Anabolic

(the "A" of GRAPE)

Front 8

GLUCOKINASE vs. HEXOKINASE

- Glucokinase is where?
- Hexokinae is where?

Back 8

- Beta cells of Pancreas
- Liver cells

- Ubiquitous
- Everywhere

Front 9

GLUCOKINASE vs. HEXOKINASE

- which has high Km ?
- which has high Vmax?

Back 9

- Glucokinase

- Glucokinase

Front 10

GLUCOKINASE vs. HEXOKINASE

- which has high Affinity?
- which as high Capacity?

Back 10

- Hexokinase

- Glucokinase

Front 11

GLUCOKINASE vs. HEXOKINASE

- which one is INSULIN-INDUCED?

Back 11

- Glucokinase

Front 12

GLUCOKINASE vs. HEXOKINASE

- these 2 enzymes do what same function?

Back 12

1st Step of Glycolysis

(Phosphorylation of Glucose to yield Glucose-6-Phosphate)

Front 13

Phosphorylation of Glucose
to yield Glucose-6-Phosphate
is the 1st step of what process(es)?

(Glucose + ATP ==> G6P)

Back 13

- Glycolysis

- Glycogen Synthesis (in the Liver)

Front 14

GLUCOKINASE vs. HEXOKINASE

- Direct Feedback Inhibition of Glucokinase?

Back 14

- none

Front 15

GLUCOKINASE vs. HEXOKINASE

- Direct Feedback Inhibition of Hexokinase?

Back 15

- Glucose-6-Phosphate

Front 16

Glucose phosphorylation by which enzyme occurs immediately AFTER MEALS?

- Where?
- Why?
- What does this allow for?

Back 16

- Glucokinase

- Liver
- Phosphorylation traps glucose in Liver

- Allows Liver to serve as a BLOOD GLUCOSE BUFFER

Front 17

GLYCOLYSIS

- Glycolysis net reaction equation

Back 17

Glucose + Pi + ADP + NAD+
==>
2 pyruvate + 2 ATP + 2 NADH + 2H2O + 2H+

Front 18

GLYCOLYSIS

- List the reactions that require ATP including the enzyme involved.

Back 18

Step 1: Glucokinase/Hexokinase
Glucose ==> Glucose-6-Phosphate

Step 3: PFK-1 (rate-limiting step)
F6P ==> F1,6-BP

Front 19

GLYCOLYSIS

- List the reactions that generate ATP including the enzyme involved.

Back 19

Step 7: PG Kinase
1,3-BPG ==> 3-PG

Step 9: Pyruvate Kinase
PEP ==> Pyruvate

Front 20

GLUCOKINASE/HEXOKINASE

- what are the inhibitor(s)
- what are the stimulator(s)

Back 20

- G6P inhibits Hexokinase
(does NOT inhibits Glucokinase)

- none

Front 21

PFK - 1

- what are the inhibitor(s)
- what are the stimulator(s)

Back 21

- Citrate
- ATP

- Fructose-2,6-BP
- AMP

Front 22

PYRUVATE KINASE

- what are the inhibitor(s)
- what are the stimulator(s)

Back 22

- Alanine
- ATP

- Fructose-1,6-BP

Front 23

PYRUVATE DEHYDROGENASE

- what are the inhibitor(s)
- what are the stimulator(s)

Back 23

- Acetyl-CoA
- NADH
- ATP

- none

Front 24

FRUCTOSE BISPHOSPHATASE 2

- active in which state?

Back 24

- Fasting state

Front 25

PHOSPHOFRUCTOKINASE 2

- active in which state?

Back 25

- Fed state

Front 26

FRUCTOSE BISPHOSPHATASE 2

- FBP-2 enzyme does what reaction?

Back 26

Fructose-2,6-BP ==> Fructose-6-P

Front 27

PHOSPHOFRUCTOKINASE 2

- PFK-2 enzyme does what reaction?

(what reaction done by PFK-1)

Back 27

Fructose-6-P ==> Fructose-2,6-BP

(F6P ==> F-1,6-BP)

Front 28

FASTING STATE vs. FEEDING STATE

- which state experiences increased Glucagon?

- Glucagon has what effect to intracellular secondary messengers?

Back 28

- Fasting state

- Increases cAMP

Front 29

FASTING STATE vs. FEEDING STATE

- which state experiences increased Insulin?

- Insulin has what effect to intracellular secondary messengers

Back 29

- Feeding state

- Decreases cAMP

Front 30

FASTING STATE

- hormone effect
- secondary messenger effect
- enzymatic effect
- effect on FBPase-2
- effect on PFK-2

Back 30

- increased Glucagon
- increases cAMP
- increases Protein Kinase A

- increases FBPase-2 activity
- decreases PFK-2 activity

Front 31

FEEDING STATE

- hormone effect
- secondary messenger effect
- enzymatic effect
- effect on FBPase-2
- effect on PFK-2

Back 31

- increases Insulin
- decreases cAMP
- decreases Protein Kinase A

- decreases FBPase-2 activity
- increases PFK-2 activity

Front 32

Fructose-2,6-Bisphosphate

- is a positive feed-forward stimulator of?

Back 32

PFK-1

(rate limiting enzyme for glycolysis)

Front 33

PFK-2

- drives what pathway?

Back 33

- Glycolysis

(b/c in fed state)

Front 34

FBPase-2

- drives what pathway?

Back 34

- Gluconeogenesis

(b/c in fasting state)

Front 35

Fructose-1,6-BisPhosphate stimulates what enzyme?

Fructose-2,6-BisPhosphate stimulates what enzyme?

Back 35

- Pyruvate Kinase

- PFK-1

Front 36

PYRUVATE DEHYDROGENASE COMPLEX

- Reaction equation

Back 36

Pyruvate + CoA + NAD
==>
Acetyl-CoA + CO2 + NADH

Front 37

PYRUVATE DEHYDROGENASE COMPLEX

- how does exercise effect it?
- exercise effects via what changes? x3

Back 37

- Activates Pyruvate DHase

Increases:
- Ca2+
- ADP
- NAD+/NADH ratio

Front 38

Alcohol metabolsim effects on Niacin based ratio.

Exercise effects on Niacin based ratio.

Back 38

Increases NADH/NAD+ ratio

Increases NAD+/NADH ratio

Front 39

PYRUVATE DEHYDROGENASE COMPLEX

- effects of Arsenic on Pyruvate DHase
- how so?

Back 39

- inhibits Pyruvate DHase

- by inhibiting cofactor Lipoic Acid (B4)

Front 40

PYRUVATE DEHYDROGENASE COMPLEX

- Arsenic poisoning symptoms

Back 40

Garlic breath

Vomiting

Rice-water stools

Front 41

PYRUVATE DHase DEFICIENCY

- Acquired or Congenital?

Back 41

- Both

Front 42

PYRUVATE DHase DEFICIENCY

- Acquired PyrDHase Deficiency seen in what population groups?

- why?

Back 42

- Alcoholics

- causes B1 deficiency
(TPP is a cofactor of PyrDHase)

Front 43

PYRUVATE DHase DEFICIENCY

- causes backup of what substrate?
- substrate backup leads to what condition?
- symptoms?

Back 43

- Pyruvate
- Alanine

- Lactic Acidosis

- Neurologic defects

Front 44

PYRUVATE DHase DEFICIENCY

- Treatment?
- give examples of treatment?

Back 44

- Increase intake of Ketogenic nutrients
(so you have less glucogenic AA)

- High Fat content diet
- Diet high in Leucine & Lycine

Front 45

List the only 2 pure Ketogenic amino acids.

Back 45

Leucine & Lycine

Front 46

Products of Pyruvate

- that goes to the Cytosol
- include the enzymes responsible

Back 46

- Lactate (Lactate DHase)
- Alanine (ALT)

Front 47

Products of Pyruvate

- that goes to the Mitochondria
- include the enzymes responsible

Back 47

- Oxaloacetate (Pyr. Carboxylase)

- Acetyl-CoA (Pyruvate DHase)

Front 48

Products of Pyruvate

- which product requires ATP (& CO2)?

Back 48

- OxaloAcetate

Front 49

Products of Pyruvate

- which product requires NAD+?

Back 49

- Acetyl-CoA

Front 50

Products of Pyruvate

- which product requires B1?

Back 50

- Acetyl-CoA

(b/c Pyr. DHase requires TPP)

Front 51

Products of Pyruvate

- which product requires NADH?

Back 51

- Lactic acid

Front 52

Products of Pyruvate

- Alanine function?

Back 52

- carries NH3 to the Liver

(from Muscle)

Front 53

Products of Pyruvate

- Oxaloacetate function?

Back 53

- Replenishes TCA

- used for Gluconeogenesis

Front 54

Products of Pyruvate

- Acetyl-CoA function?

Back 54

- Transitions to TCA

Front 55

Products of Pyruvate

- Lactic acid function?

Back 55

- End product of Anaerobic glycolysis

Front 56

Products of Pyruvate

- Anaerobic glycolysis is the major pathway for what cells?

Back 56

- RBC
- WBC
- Renal MEDULLA

- Testes
- Lens
- Cornea

Front 57

TCA cycle

- produces what side products?

Back 57

1 GTP
1 FADH2

2 CO2
3 NADH

Front 58

TCA cycle

- produces how much ATP per Acetyl-CoA

- produces how much ATP per Glucose

Back 58

- 12 ATP

- 24 ATP

Front 59

TCA cycle

- Pyruvate DHase Inhibitors
- Pyruvate DHase Stimulators

Back 59

- ATP
- NADH
- Acetyl-CoA

- none

Front 60

TCA cycle

- Citrate Synthase Inhibitors
- Citrate Synthase Stimulators

Back 60

- ATP

- none

Front 61

TCA cycle

- Isocitrate DHase Inhibitors
- Isocitrate DHase Stimulators

Back 61

- ATP
- NADH

- ADP

Front 62

TCA cycle

- Alpha Ketoglutarate DHase Inhibitors
- Alpha Ketoglutarate DHase Stimulators

Back 62

- ATP
- NADH
- Succinyl-CoA

- none

Front 63

TCA cycle

- what reaction forms GTP?

Back 63

Succinyl-CoA ==> Succinate

Front 64

TCA cycle

- what reaction forms FADH2?

Back 64

Succinate ==> FORMATE

Front 65

TCA cycle

- what reaction forms NADH?

Back 65

Isocitrate ==> Alpha-ketoglutarate

Alpha-ketoglutarate ==> Succinyl-CoA

Malate ==> Oxaloacetate

Front 66

Pyruvate (via Pyr. DHase) forms Acetyl-CoA along with what other products?

Back 66

1 NADH

1 CO2

Front 67

TCA cycle

- which TCA cycle enzyme is Complex II of the ETC?

Back 67

- Succinate DHase

Front 68

TCA cycle

- NADH electrons from Glycolysis & TCA enter ______ via _________ and ________

Back 68

- Mitochondria

- Malate-Aspartate shuttle
- Glycerol-6-Phosphate shuttle

Front 69

TCA cycle

- FADH2 electrons from TCA are transferred to which ETC complex?

Back 69

- Complex II

Front 70

ETC & Ox Phos

- Protons enter the Mitochondrial Matrix via what complex?

Back 70

Complex V

(ATP synthase)

Front 71

ETC & Ox Phos

- Protons enter the Intermembranous space via what complex?

Back 71

Complex I, III, & IV

Front 72

GLUCONEOGENESIS

- what are the IRREVERSIBLE enzymes?

Back 72

(Pathway Producing Fresh Glucose)

- Pyruvate Carboxylase
- PEP Carboxykinase
- Fructose-1,6-Bisphosphatase
- Glucose-6-Phosphate

Front 73

GLUCONEOGENESIS

- which irreversible enzyme is in the mitochondria?

- which is in the cytosol?

- which is in the ER?

Back 73

- Pyruvate Carboxylase

- PEP Carboxykinase
- Fructose-1,6-Bisphosphatase

- Glucose-6-Phosphate

Front 74

GLUCONEOGENESIS

- which enzyme requires ATP?
- which enzyme requires GTP?

Back 74

- Pyruvate Carboxylase
(also requires Biotin)

- PEP Carboxykinase

Front 75

GLUCONEOGENESIS

- which enzyme requires Acetyl-CoA to become activated?

Back 75

- Pyruvate Carboxylase

Front 76

GLUCONEOGENESIS

- occurs primarily where?
- deficiency causes what plasma changes?

Back 76

- Liver

- Hypoglycemia

Front 77

GLUCONEOGENESIS

- what tissues can NOT participate in Gluconeogenesis?
- why?

Back 77

- Muscle tissue

- lacks Glucose-6-Phosphatase

Front 78

GLUCONEOGENESIS

- which fatty acids can NOT participate in Gluconeogenesis?

- why?

Back 78

- Even-chain FA

- b/c they yield only Acetyl-CoA equivalents

Front 79

GLUCONEOGENESIS

- Odd-chain FA yields what during metabolism?

- these can enter what pathway?
- enters pathway as what?

Back 79

- Propionyl-CoA

- TCA
- as Succinyl-CoA
(then undergo gluconeogenesis)

Front 80

HMP SHUNT

- NADPH comes from what intermediate?

Back 80

- G6P

Front 81

HMP SHUNT

- besides NADPH, this HMP also provides what compound?

- above compound is useful for?

Back 81

- Ribose

- Nucleotide synthesis
- Glycolysis intermediates

Front 82

HMP SHUNT

- how much ATP is produced?
- how much ATP is used up?

Back 82

- none

- none

Front 83

HMP SHUNT

- occurs where? x4

Back 83

- RBC
- Adrenal Cortex
- Liver
- Lactating mammary glands

Front 84

HMP SHUNT

- Rate limiting enzyme?

Back 84

- G6PDHase

Front 85

HMP SHUNT

- Oxidative phase enzyme?
- Oxidative phase substrate?
- Oxidative phase products?

Back 85

- G6PDHase

- G6P

- 2 NADPH
- Ribulose
- CO2

Front 86

HMP SHUNT

- Non-oxidative phase enzyme?
- Non-oxidative substrate?
- Non-oxidative products?

Back 86

- Transketolase (B1 cofactor)

- Ribulose

- G3P
- F6P
- Ribose-5-P

Front 87

HMP SHUNT

- which phase is irreversible?

Back 87

- Oxidative phase

Front 88

RESPIRATORY BURST

- involves the activation of what enzyme?

- enzyme located where?

Back 88

- NADPH Oxidase

- Membrane surface of RBC

Front 89

RESPIRATORY BURST

- results in the rapid release of what?

Back 89

Reactive Oxygen Intermediates (ROIs)

Front 90

RESPIRATORY BURST

- NADPH Oxidase deficiency is called what?

Back 90

- Chronic Granulomatous Disease (CGD)

Front 91

Patients with CGD are at increased risk for infection by what TYPE of species?

- give examples

- why are these species so effective in infecting CGD patients?

Back 91

- Catalase Positive

- Staph. Aureus
- Aspergillus

- Catalase positive species can neutralize their own H2O2, thus WBCs cannot make ROIs to fight infection

Front 92

G6PDHase Deficiency

- NADPH is necessary to keep Glutathione _____, because it will detoxify ______ & _________.

Back 92

- Reduced

- Free Radicals
- Peroxides

Front 93

G6PDHase Deficiency

- Decreased NADPH in RBCs lead to what type of anemia?

- this is due to poor RBC defense against what agents?

- list some of these agents

Back 93

- Hemolytic anemia

- Oxidizing agents

- Fava beans
- MTX
- INH
- Sulfonamides
- TB-drugs

Front 94

G6PDHase Deficiency

- inheritance pattern?
- more commonly seen in what population group?
- diseased patients have a unique resistance to what other disease?

Back 94

- XLR

- Blacks

- Malaria

Front 95

G6PDHase Deficiency

- what type of cells do you see on blood smears?

- what causes their morphology?

Back 95

Heinz Bodies
- oxidized Hb precipitated on RBC

Bite Cells
- "bite" removed from phagocytic removal of Heinz bodies by macrophages

Front 96

Essential Fructosuria

- etiology?
- inheritance pattern?
- Sx?

Back 96

- Fructokinase defect

- AR

- mild symptoms with Fructose found in blood & urine

Front 97

Fructose Intolerance

- etiology
- inheritance pattern?

Back 97

- Aldolase B deficiency

- AR

Front 98

Fructose Intolerance

- what substrate accumulates?
- thus cause what electrolyte change?
- thus leading to what effects on pathways?

Back 98

- Fructose-1-Phosphate

- Decrease in Phosphate

Inhibition of:
- Gluconeogenesis
- Glycogenolysis

Front 99

Fructose Intolerance

- Sx x4

Back 99

- Hypoglycemia
- Jaundice
- Cirrhosis
- Vomiting

Front 100

Fructose Intolerance

- Tx?

Back 100

Decrease intake of:
- Fructose
- Sucrose

(note Sucrose = Glucose + Fructose)

Front 101

Fructose is not glucose.
Fructose metabolism bypass the glycolytic pathway by bypassing with glycolytic step?

via what enzyme?

Back 101

- Rate limiting step involving PFK-1

- aldolase B

Front 102

Back 102

Front 103

Back 103

Front 104

Galactokinase Deficiency results in accumulation of ________ , but only if __________ .

This occurs due to the alternative pathway involving what enzyme?

Back 104

- Galactitol

- Galactose is in the diet

- Aldol Reductase

Front 105

Galactokinase Deficiency

- what changes are found in plasma?
- what changes are found in urine?

Back 105

- Increased Galactose
(increased galactitol if galactose in diet)

- Increased Galactose

Front 106

Galactokinase Deficiency

- most notable symptom in infants
- may initially present as infant failure to do what?

Back 106

- Infantile Cataracts

- Failure to track objects
- Failure to develop Social Smile

Front 107

Classic Galactosemia

- etiology?
- damage is caused by accumulation of toxic substances including ____ which accumulates in _________.

Back 107

- Galactose-1-Phosphate Uridyl Transferase

- Galactitol
- Lens of the eye

Front 108

Classic Galactosemia

- symptoms

Back 108

- Infantile cataracts
- MR
- Jaundice
- Hepatosplenomegaly
- FTT

Front 109

Classic Galactosemia

- Tx

Back 109

EXCLUDE
- Galactose
- Lactose

Note: Lactose = Galactose + Glucose

Front 110

Alternative method of trapping glucose in the cell is to covert it to?

Occurs via what enzyme?

Back 110

- Sorbital
(alcohol counterpart)

- Aldol Reductase

Front 111

Some cells can convert Sorbital to ______, using the enzyme __________.

Back 111

- Fructose

- Sorbitol DHase

Front 112

ALDOL REDUCTASE

- converts what reaction?
- requires what cofactor?

Back 112

Glucose ==> Sorbitol

NADPH

Front 113

ALDOL REDUCTASE

- what cells only have this enzyme, but lack Sorbital DHase?

Back 113

- Schwann cells
- Kidney cells
- Retina
- Lens

Front 114

SORBITAL DEHYDROGENASE

- converts what reaction?
- requires what cofactor?

Back 114

Sorbital ==> Fructose

NAD+

Front 115

SORBITAL DEHYDROGENASE

- cells lacking this enzyme are at risk for INTRACELLULAR accumulation of?

- what TYPE of Damage is seen?

- give examples of symptoms that can arise?

Back 115

- Sorbital

- Osmotic damage

- Cataracts
- Retinopathy
- Peripheral Neuropathy

(Sx seen in chronic hyperglycemia of DM patients)

Front 116

Back 116

Front 117

What are the Essential Amino Acids?

(x10)

Back 117

(PVT TIM HALL)

- Phenylalanine
- Valine
- Threonine

- Tryptophan
- Isoleucine
- Methionine

- Histidine
- Alanine
- Lycine
- Leucine

Front 118

Which Essential Amino acids are purely Ketogenic? (x2)

Which Essential Amino acids are both Ketogenic & Glucogenic? (x4)

Which Essential Amino acids are purely Glucogenic? (x4)

Back 118

- Lysine & Leucine

(PITT)
- Phenylalanine
- Isoleucine
- Threonine
- Tryptophan

- Histidine
- Alanine
- Methionine
- Valine

Front 119

- Which amino acids are Acidic?

- Which amino acid is negatively charged at body pH)

Back 119

- Aspartate (Aspartic acid)
- Glutamate (Glutamic acid)

- Glutamate

Front 120

- Which amino acids are Basic?

- Which amino acid is most basic?

- Which amino acid has no charge at body pH?

Back 120

- Lysine
- Arginine
- Histidine

- Arginine

- Histidine

Front 121

Which amino acids are required during periods of growth?

Back 121

- Histidine

- Arginine

Front 122

Which amino acid is NEGATIVELY CHARGED at body pH?

Back 122

- Glutamate

Front 123

Which amino acid is NEUTRAL at body pH?

Back 123

- Histidine

Front 124

UREA CYCLE

- rate-limiting step reaction?
- rate-limiting enzyme?
- additionally required reactants?
- occurs in what organelle?
- occurs in what cell?

Back 124

NH4 + CO2 ==> Carbomoyl Phosphate

Carbamoyl Phosphate Synthetase I

2 ATP

Mitochondria

Liver

Front 125

UREA CYCLE

- what compound leaves the liver mitochondria and goes to the liver cytoplasm?

Back 125

- Citrulline

Front 126

UREA CYCLE

- what compound leaves the liver cytoplasm and goes into the liver mitochondria?

Back 126

- Ornithine

Front 127

UREA CYCLE

- what intermediate leaves the mitochondria?
- what is the reaction that forms it?
- what is the enzyme involved?

Back 127

- Citrulline

Carbamoyl-P + Ornithine ==> Citrulline

- Ornithine Transcarbamoylase

Front 128

UREA CYCLE

- excess nitrogen (NH4+) is finally converted into what?

- which then goes where?
- for what?

Back 128

- Urea

- Kidney

- Excretion

Front 129

AMMONIUM

- Ammonia (NH3) from muscle comes from what?

- Ammonia (NH3) from muscle is transported to what organ?

- Ammonia (NH3) from muscle is transported in blood via what intermediate?

Back 129

- Amino acids

- Liver

- Alanine

Front 130

AMMONIUM

- Ammonium (NH3) from muscle is transported to the Liver as what intermediate?

- In the Liver, the Ammonium (NH3) is finally incorporated into what chemical compound?

Back 130

- Alanine

- Urea

Front 131

HYPERAMMONEMIA

- excess NH4+ causes depletion of what intermediate?

- this leads to inhibition of what pathway?

Back 131

- Alpha-Ketoglutarate

- TCA

Front 132

HYPERAMMONEMIA

- Ammonia intoxication causes what symptoms.

Back 132

- Blurry vision
- Cerebral edema
- Tremor
- Vomiting
- Slurring speech
- Somnelence

Front 133

HYPERAMMONEMIA

- First Tx option?
- In order to decrease Ammonia levels, what medications can be given?

Back 133

- Limit Protein in Diet

- Benzoic acid
- Phenylbutyrate

Front 134

OTC DEFICIENCY

- inheritance pattern
- excess intermediate?
- excess intermediate is converted into what?

Back 134

- XLR

- Carbamoyl Phosphate

- Orotic acid
(part of pyrimidine synthesis pathway)

Front 135

OTC DEFICIENCY

- what is elevated in blood?
- what is decreased in blood?

Back 135

- Orotic acid
(also in urine)

- BUN

Front 136

OTC DEFICIENCY

- what are the symptoms of OTC Deficiency?

Back 136

Symptoms of Hyperammonemia
- Blurry vision
- Cerebral edema
- Tremors
- Vomiting
- Slurring speech
- Somnelence

Front 137

OTC DEFICIENCY

- interferes with the body's ability to do what?

Back 137

- eliminate ammonia

Front 138

Back 138