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Biochem Flashcards

1
Q

Only secondary AA

A

proline

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2
Q

Glucogenic essential AA

A

Met
Val
His

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3
Q

Ketogenic essential AA

A

Leu

Lys

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4
Q

Glucogenic/Ketogenic essential AA

A

Isoleucine
Phenylalanine
Threonine
Tyrptophan

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5
Q

Charge of acidic AA at body pH

A

negative

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6
Q

Acidic AA

A

Asp

Glu

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7
Q

Charge of basic AA at body pH

A

Positive

His has NO charge

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8
Q

Basic AA

A

Arg
Lys
His
(arg and lys are high in histones to bind negatively charged DNA)

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9
Q

Which AAs have 3 titratable protons?

A

Acidic AA (Asp, Glu)
Basic AA (Arg, Lys, His)
Cysteine
Tyrosine

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10
Q

Only urea cycle disorder that is X-linked recessive

A

ornithine transcarbamylase deficiency

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11
Q

AA used NO, Creatinine, Urea

A

Arginine

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12
Q

AA used to make GABA and Glutathione

A

Glutamate

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13
Q

AA used to make Heme

A

GLycine

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14
Q

AA used to make Histamine

A

Histidine

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15
Q

AA used to make Serotonin, Melatonin, Niacin, and NAD+/NADP+

A

Tryptophan

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16
Q

AA used to make Thyroxine, Melanin, Dopamine, NE, and Epi

A

Phenylalanine

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17
Q

What is required in the step from phenylalanine to tyrosine?

A

BH4 (regenerated from BH2 by dihydropiopterin reductase)

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18
Q

What is required in the step from Tyrosine to Dopa?

A

BH4

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19
Q

What is required in the step from dopa to Dopamine?

A

Vitamin B6

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20
Q

What is required in the step from Dopamine to NE?

A

Vitamin C (Dopamine hydoxylase)

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21
Q

What is required in the step form NE to Epi?

A

SAM

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22
Q

What vitamin is needed to synthesize niacin form tryptophan?

A

B6

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23
Q

What is needed to make 5-HT from Tryptophan?

A

BH4 and B6

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24
Q

What other amino acids need B6 to make their products?

A
  • Phenalanine (Dopa to DA)
  • Tryptophan (to niacin and to 5-HT)
  • Histidine (to histamine)
  • Glutamate (to GABA)
  • Glycine (to porphyrin)
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25
Q

What is needed to make NO from arginine?

A

BH4

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26
Q

What enzyme is deficient in PKU?

A

Phenylalanine hydroxylate

or tetrahydrobiopterin cofactor

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27
Q

What builds up in PKU?

A

phenylalanine

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28
Q

How do you treat PKU?

A

low phenylalanine, high tyrosine diet

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29
Q

What enzyme is deficient in Alkaptonuria?

A

Homogentisate oxidase

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30
Q

What builds up in alkaptonuria?

A

Homogentisic Acid (toxic to joints)

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31
Q

What are the 3 causes of homocystinuria?

A
  • Cysthathionine synthase deficiency
  • Decreased affinity of cystathionine synthase for pyridoxal phosphate
  • Homocysteine methyltransferase deficiency
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32
Q

How do you treat cystathionine synthase deficiency?

A

Methionine will be high and cysteine will be low, so decrease methionine and increase cysteine and B12/folate in the diet

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33
Q

How do you treat a decreased affinity of cystathionine synthase for pyridoxal phosphate

A

Give extra B6 and increase cysteine in the diet

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34
Q

How do you treat a homocysteine methyltransferase deficiency?

A

Methionine will be low, so increase it in the diet

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35
Q

What causes cystinuria?

A

defect in AA transporter in renal PCT for Cysteine, Ornithine, Lysine and Arginine (COLA)

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36
Q

What causes MSUD?

A

decreased alpha-ketoacid dehydrogenase (B1= thiamine is cofactor)

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37
Q

What builds up in MSUD?

A

branched amino acids (isoleucine, leucine and valine)

38
Q

What is “tender love and care for Nancy”?

A
  • Thiamine pyrophosphate
  • Lipoate
  • CoA
  • FAD
  • NAD
39
Q

What enzymes need tender love and care for Nancy?

A
  • Alpha-ketoacid dehydrogenase
  • Alpha-ketoglutarate dehydrogenase
  • Pyruvate dehydrogenase
40
Q

CT stain

41
Q

Muscle stain

42
Q

Epithelial cell stain

A

Cytokeratin

43
Q

Neuroglia stain

44
Q

Neuron stain

A

neurofilaments

45
Q

Rate limiting step of glycolysis (and regulators)

A

PFK-1

increased by AMP and fructose-2,6-bisphosphate; decreased by ATP and citrate

46
Q

Rate limiting step of gluconeogenesis (and regulators)

A

Fructose-1,6-Bisphosphate

increased by ATP and acetyl-CoA; decreased by AMP and fructose-2,6-bisphosphate

47
Q

Rate limiting step of TCA cycle (and regulators)

A

Isocitrate dehydrogenase

increased by ADP; decreased by ATP and NADH

48
Q

Rate limiting step of glycogenesis (and regulators)

A

Glycogen synthase

increased by G-6-P, insulin and cortisol; decreased by epi and glucagon

49
Q

Rate limiting step of glycogenolysis (and regulators)

A

Glycogen phosphorylase

increased by epi and glucagon; decreased by G-6-P, insulin and ATP

50
Q

Rate limiting step of HMP shunt (and regulators)

A

G6PD

increased by NADP+; decreased by NADPH

51
Q

Rate limiting step of De novo pyrimidine synthesis

A

Carbamoyl phosphate synthetase II (needs 2 ATP)

52
Q

Rate limiting step of De novo purine synthesis (and regulators)

A

Glutamine-PRPP amidotransferase

decreased by AMP, IMP and GMP

53
Q

Rate limiting step of urea cycle (and regulators)

A

Carbamoyl phosphate synthetase I

increased by N-acetylglutamate

54
Q

Rate limiting step of fatty acid synthesis (and regulators)

A

Acetyl-CoA-Carboxylase

increased by insulin and citrate; decreased by glucagon and palmitoyl-CoA

55
Q

Rate limiting step of fatty acid oxidation (and regulators)

A

Carnitine acyltransferase I

decreased by malonyl-CoA

56
Q

Rate limiting step of cholesterol synthesis (and regulators)

A

HMG-COA reductase

increased by insulin and thyroxine; decreased by cholesterol and glucagon

57
Q

Rate limiting step of ketogenesis

A

HMG-CoA synthase

58
Q

What is deficient in Von Gierke disease?

A

Glucose 6 Phosphate (can’t convert G-6-P back to glucose during glycogenolysis)

59
Q

What is deficient in Pompe disease?

A

Lysosomal alpha-1,4-glucosidase (acid maltase)

60
Q

What is deficient in Cori Disease?

A

Debranching enzyme alpha-1,6-glucosidase (can’t remove last residue)

61
Q

What is deficient in McArdle disease?

A

Skeletal muscle glycogen phosphorylase (myophosphorylase) so can’t get branching glycogen down to 4 untis

62
Q

Which glycogen storage disease leads to cardiomyopathy?

A

Pompe disease

63
Q

Which glycogen storage disease leads to painful muscle cramps and myogloginuria with strenuous exercise?

A

McArdle disease

64
Q

Which glycogen storage disease leads to severe fasting hypoglycemia and increased glood lactate with hepatomegaly (increased glycogen in liver)?

A

Von Gierke disease

65
Q

Which glycogen storage is a mimic of Von Gierke (but normal blood lactate levels)?

A

Cori disease

66
Q

Which lysosomal storage disease is not AR?

A

Fabry disease (XLR)

67
Q

What is deficient in Fabry?

A

Alpha-galactosidase A

68
Q

What builds up in Fabry?

A

Ceramide trihexoside

69
Q

What is deficient in Gaucher?

A

Glucocerebroside (Beta-glucosidase)

70
Q

What builds up in Gaucher?

A

Glucocerebroside

71
Q

What is deficient in Neimann-Pick?

A

Sphingomyelinase

72
Q

What builds up in Neimann-Pick?

A

Sphingomyelinase

73
Q

What is deficient in Tay Sachs?

A

Hexosaminidase A

74
Q

What builds up in Tay Sachs?

A

GM2 ganglioside

75
Q

What is deficient in Krabbe disease?

A

Galactocerebrosidase

76
Q

What builds up in Krabbe disease?

A

Galactocerebroside, psychosine

77
Q

What is deficient in metachromatic leukodystrophy?

A

Arylsulfatase A

78
Q

What builds up in metachromatic leukodystrophy?

A

Cerebroside sulfate

79
Q

What is deficient in Hurler?

A

alpha-L-iduronidase

80
Q

What builds up in Hurler?

A

Heparin Sulfate; dermatan sulfate

81
Q

What is deficient in Hunter?

A

Iduronate sulfatase

82
Q

What builds up in Hunter?

A

Heparin Sulfate; dermatan sulfate

83
Q

LSD causing peripheral neuropathy, developmental delay, optic atrophy and globoid cells

A

Krabbe disease

84
Q

LSD causing progressive neurodegeneration, hepatosplenomegaly, “cherry-red” spot on macula, foam cells

A

Neimann-Pick

85
Q

LSD causing Hepatosplenomegaly, pancytopenia, bone crises, aseptic necrosis of femur, crumpled tissue paper macrophages

A

Gaucher disease

86
Q

LSD causing progressive neurodegeneration, developmental delay, “cherry red” spot on macula, lysosomes with onion skin

A

Tay Sachs (NO HEPATOSPLENOMEGALY)

87
Q

LSD causing peripheral neuropathy of hands/feet, angiokeratomas, CV/renal disease

A

Fabry disease

88
Q

LSD causing aggressive behavior and no corneal clouding with some developmental delay, gargolylism, airway obstruciton and hepatosplenomegaly

A

Hunter syndrome

89
Q

LSD causing central and peripheral demyelination with ataxia and dementia

A

Metachromatic leukodsytrophy

90
Q

LSD causing developmental delay, gargoylism, airway obstruciton, corneal clouding and hepatosplenomegaly

A

Hurler syndrome

91
Q

What leads to inability to transport LCFAs into mitochrondria?

A

Carnitine deficiency

92
Q

What are the symptoms of carnitine deficiency?

A 
Weakness
Hypotonia
Hypoketoid hypoglycemia
myoglobinemia
Increase muscle TGs

1st Aid Memorize (21 decks)

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