Biochemistry Med Essentials

Front 1

Glycolysis occurs where

Back 1

Cytoplasm

Front 2

In glycolysis 1 glucose is converted to _ pyruvates, net of _ ATP's by substrate level phosphorylation and _ NADH's

Back 2

2
2
2

Front 3

3 irreversible steps of glycolysis

Back 3

Hexokinase/glucokinase
PFK1
Pyruvate kinase

Front 4

Happens in most tissues

a)glucokinase
b)hexokinase

Back 4

Hexokinase

Front 5

Happens only in liver

a)glucokinase
b)hexokinase

Back 5

Glucokinase

Front 6

Which has low Km

a)glucokinase
b)hexokinase

Back 6

Hexokinase

Front 7

Which has high Km

a)glucokinase
b)hexokinase

Back 7

Glucokinase

Front 8

Inhibits hexokinase

Back 8

G-6-P

Front 9

Stimulates glucokinase

Back 9

Insulin

Front 10

Rate limiting step of glycolysis

Back 10

Conversion of F-6-P to F-1,6-BP by PFK1

Front 11

_ and _ stimulate PFK1

Back 11

AMP and F 2,6 BP

Front 12

_ and _ inhibit PFK1

Back 12

Citrate and ATP

Front 13

Glycolysis regulator - increases glycolysis, decreases gluconeogenesis

Back 13

PFK2

Front 14

PFK 2 is stimulated by _ and inhibited by _

Back 14

insulin
glucagon

Front 15

_ and _ stimulate pyruvate kinase

Back 15

F1,6 BP and insulin

Front 16

4 things that inhibit pyruvate kinase

Back 16

ATP
Acetyl CoA
Alanine
Glucagon

Front 17

Glucose transport - basal uptake in most cells

a)GLUT 1 + GLUT3
b)GLUT2
c)GLUT4

Back 17

GLUT1 + GLUT3

Front 18

_ mutations may lead to form of MODY

Back 18

Glucokinase

Front 19

Glucose transporter - storage (liver), glucose sensor (beta islet)

a)GLUT 1 + GLUT3
b)GLUT2
c)GLUT4

Back 19

GLUT2

Front 20

Glucose transporter

Increased by insulin (fat and muscle), increased by exercise (skeletal muscle)

a)GLUT 1 + GLUT3
b)GLUT2
c)GLUT4

Back 20

GLUT4

Front 21

Galactosemia/galactosuria, cataracts in childhood

Tx = no galactose in diet

Whats defficient

Back 21

Galactokinase

Front 22

Galactosemia/galactosuria, cataracts in childhood, vomitting/diarrhea after milk digestion, liver disease, lethargy, MR

Tx - no galactose in diet

Whats defficient

Back 22

Gal-1-P uridyl transferase

Front 23

Excess galactose is converted to galactitol via _

Back 23

Aldose reductase

Front 24

Benign fructosuria - whats defficient

Back 24

Fructokinase

Front 25

Fructosuria, liver and proximal renal tubule disorder

Tx = no fructose in diet

Whats defficient

Back 25

Aldolase B defficiency

Front 26

Chronic hemolysis, increased 2,3 BPG and other glycolytic intermediates in RBC, no Heinz bodies, AR

Whats defficient

Back 26

Pyruvate kinase

Front 27

TCA occurs where

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Mitochondria

Front 28

Can TCA occur under anaerobic conditions

Back 28

NO

Front 29

Each acetyl CoA generated from pyruvate in TCA is used to ptoduce _ NADH, _ FADH2 and _ GTP

Back 29

3
1
1

Front 30

3 regulatory enzymes of TCA cycle

Back 30

Citrate synthase
Isocitrate dehydrogenase
Alpha ketoglutarate dehydrogenase

Front 31

TCA is linked to gluconeogenesis through _

Back 31

Malate shuttle

Front 32

TCA is linked to FA synthesis through _

Back 32

Citrate

Front 33

TCA is linked to amino acid synthesis through _

Back 33

Oxaloacetate and Alpha KG

Front 34

TCA is linked to heme synthesis through _

Back 34

Succinyl CoA

Front 35

Inhibits citrate synthase

Back 35

ATP

Front 36

Rate limiting step of TCA

Back 36

Isocitrate dehydrogenase

Front 37

Stimulates isocitrate dehydrogenase

Back 37

ADP

Front 38

Inhibits isocitrate dehydrogenase (2)

Back 38

ATP
NADH

Front 39

Inhibits Alpha KG dehydrogenase (3)

Back 39

Succinyl CoA
NADH
ATP

Front 40

ETC occurs where

Back 40

Inner mitochondrial membrane

Front 41

In ETC there are _ ATP's per NADH and _ ATP's per FADH2

Back 41

3
2

Front 42

How do we treat CN poisoning

Back 42

Nitrites - creates methemoglobin which binds CN

Front 43

Complex I of ETC is _

Back 43

NADH dehydrogenase

Front 44

Complex II of ETC is _

Back 44

Succinate dehydrogenase

Front 45

Complex III of ETC is _

Back 45

Cytochrome b

Front 46

Complex IV of ETC is _

Back 46

Cytochrome a

Front 47

CO inhibits what part of ETC

Back 47

Cytochrome a (complex IV)

Front 48

Antimycin inhibits what part of ETC

Back 48

Cytochrome b(complex III)

Front 49

Doxorubicin inhibits what part of ETC

Back 49

Coenzyme Q (between complex II and III)

Front 50

Oligomycin inhibits what part of ETC

Back 50

Complex V (F0)

Front 51

Name 2 uncouplers of ETC

Back 51

High dose aspirin
2,4 DNP

Front 52

Rotenone inhibits what part of ETC

Back 52

Complex I (NADH dehydrogenase)

Front 53

Cytosolic electrons transported into mitochondria via 2 carriers - name

Back 53

MALATE (from TCA) and glycerol-3-P (from DHAP in glycolysis)

Front 54

Name 4 fates of pyruvate

Back 54

Pyruvate - lactate
Pyruvate -acetyl CoA
Pyruvate -oxaloacetate
Pyruvate - alanine

Front 55

In anaerobic tissues this enzyme converts pyruvate to lactate reoxidizing NADH to NAD+, in liver it converts lactate to pyruvate for gluconeogenesis or for metabolism to acetyl CoA

Back 55

LACTATE DEHYDROGENASE

Front 56

In muscle this enzyme converts pyruvate to alanine to transport amino groups to liver and in liver it converts alanine to pyruvate for gluconeogenesis and delivers amino group for urea synthesis

Back 56

ALANINE AMINOTRANSFERASE (ALT, GPT)

Front 57

This enzyme produces oxaloacetate for gluconeogenesis and TCA

Back 57

Pyruvate carboxylase

Front 58

This enzyme generates acetyl CoA for FA synthesis and TCA

Back 58

PYRUVATE DEHYDROGENASE

Front 59

Name 4 stimulators of pyruvate dehydrogenase

Back 59

ADP
CoA
NAD
Insulin

Front 60

3 inhibitors of pyruvate dehydrogenase

Back 60

Acetyl CoA
ATP
NADH

Front 61

Lactic acidosis, seizures, MR, ataxia, spasticity - what enzyme defficient

Back 61

Pyruvate dehydrogenase

Front 62

During fasting or exercise lactate from RBC or skeletal muscle is sent to liver to make glucose that can be returned to muscle or RBC - this is called?

Back 62

CORI CYCLE

Front 63

HMP shunt (pentose phosphate pathway) occurs where

Back 63

CYTOSOL

Front 64

HMP shunt uses G-6-P to make _ and _

Back 64

NADPH and RIBOSE-5-P

Front 65

Enzyme that regulates HMP shunt

Back 65

G-6-P dehydrogenase

Front 66

_ stimulates G6P dehydrogenase

Back 66

NADP +

Front 67

_ inhibits G6P dehydrogenase

Back 67

NADPH

Front 68

Episodic hemolytic anemia induced by infection and drugs (common) or chronic hemolysis (rare), X linked recessive, female heterozygotes have increased resistance to malaria

Back 68

G6PD defficiency

Front 69

Glycogen synthase in liver is stimulated by _ and _ and in muscle only by _

Back 69

Insulin + glucose

Muscle - insulin only

Front 70

Glycogen synthase is inhibits by _ and _ in liver and by _ in muscle

Back 70

Glucagon + epinephrine

Epinephrine in muscle

Front 71

This enzyme hydrolyzes alpha 1,4 bond in growing glycogen chain then transfers oligosaccharide unit to new position and attaches it with alpha 1,6 bond to create a branch - glycogen synthase then extends both branches

Back 71

BRANCHING ENZYME

Front 72

Hydrolyzes alpha 1,4 bond closes to branch point, transfers oligosaccharide to end of another chain then hydrolyzes alpha 1,6 bond releasing single glucose remaining at branch point

Back 72

DEBRANCHING ENZYME

Front 73

Severe hypoglycemia, lactic acidosis, hepatomegaly, hyperlipidemia, hyperuricemia and short stature

a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 73

Von Gierke

Front 74

Cardiomegaly, muscle weakness, death by 2 years old


a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 74

Pompe

Front 75

Mild hypoglycemia, liver enlargement


a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 75

Cori disease

Front 76

Infantile hypotonia, cirrhosis, death by 2 years


a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 76

Andersen disease

Front 77

Muscle cramps/weakness during initial phase of exercise, possible rhabdomyolysis and myoglobinuria


a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 77

McArdle disease

Front 78

Mild fasting hypoglycemia, hepatomegaly, cirrhosis


a)von Gierke - glucose-6-phosphatase
b)Pompe - lysosomal alpha 1,4 glucosidase
c)Cori disease - glycogen debranching enzyme
d)Andersen disease - branching enzyme
e)McArdle disease - muscle glycogen phosphorylase
f)Hers disease - hepatic glycogen phosphorylase

Back 78

Hers disease

Front 79

Gluconeogenesis occurs in which body tissues

Back 79

Liver
Kidney
Intestinal epithelium

Front 80

4 irreversible steps of gluconeogenesis

Back 80

Pyruvate carboxylase
PEPCK
Fructose 1,6 bisphosphatase
Glucose 6 phosphatase

Front 81

First step of gluconeogenesis - conversion of pyruvate to _ occurs in _

Back 81

Oxaloacetate
Mitochondria

Front 82

Mitochondrial regulatory enzyme of gluconeogenesis, requires biotin

What stimulates it

Back 82

Pyruvate carboxylase

Acetyl CoA

Front 83

Cytosoic regulatory enzyme of gluconeogenesis, requires GTP

What stimulates it (2)

Back 83

PEPCK

Glucagon+ cortisol

Front 84

Glucose 6 phosphatase occurs only where

Back 84

Liver - in ER

Front 85

Fructose 1,6 bisphospatase is stimulated by _ and inhibited by _ (2)

Back 85

ATP


AMP + F-2,6 BP from PFK2

Front 86

Name nonpolar aliphatic amino acids (6)

HINT VIP GAL

Back 86

Valine
Isoleucine
Proline

Glycine
Alanine
Leucine

Front 87

Name amino acids with aromatic side chains (3)

Back 87

Phe
Tyr
Trp

Front 88

Name amino acids that are positively charged (3)

Back 88

Lys
Arg
His

Front 89

Name amino acids that are polar, uncharged (6)

GAS
MTC

Back 89

Glutamine
Asparagine
Serine

Methionine
Threonine
Cysteine

Front 90

Name negatively charged amino acids (2)

Back 90

Asparate
Glutamate

Front 91

Patient with acute abdominal pain, anxiety, confusion, paranoia, muscle weakness, no photosensitivity, port wine urine in some patients

Whats defficient

What accumulates in urine

Inheritance

Back 91

Uroporphyrinogen synthase I

Acute intermittent porphyria

ALA and porphobillinogen

AD

Front 92

Photosensitivity, skin inflammation and blistering, cirrhosis- type of porphyria, whats defficeint

Back 92

Porphyria cutanea tarda - uroporphyrinogen decarboxylase defficiency

Front 93

In lead poisoning what two enzymes are defficient

Back 93

ALA dehydratase
Ferrochelatase

Front 94

Name 2 ketogenic amino acids

Back 94

Leucine
Lysine

Front 95

Name 5 glucogenic + ketogenic acids

HINT PITTT

Back 95

Phe
Isoleucine
Trp
Tyr
Thr

Front 96

How do you differentiate carbamoyl phosphate synthase defficiency from ornithine transcarbamoylase defficiency

Back 96

In carbamoyl phosphate = no increase in uracil or orotic acid

In ornithine transcarbamoylase - increased uracil and orotic acid in blood and urine