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tsomkapQ > Biochemistry COPY > Flashcards

Biochemistry COPY Flashcards

1
Q

What is the most common extracellular buffer?

A

Bicarb

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2
Q

What is the Isoelectric Point?

A

The pH at which there is no net charge

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3
Q

What is the rate-limiting enzyme of Glycolysis?

A

PFK-1 (Phosphofructokinase-1)

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4
Q

What is the rate-limiting enzyme in Gluconeogenesis?

A

Fructose - 1,6 Bisphophatase

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5
Q

What is the rate-limiting enzyme in the HMP shunt?

A

Glucose-6-Phosphate Dehydrogenase (G-6PD)

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6
Q

What is the rate-limiting enzyme in Glycogenesis?

A

Glycogen Synthase. Occurs in the Cytoplasm of Liver and muscle

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7
Q

What is the rate-limiting enzyme in Glycogenolysis?

A

Glycogen Phosphorylase. Occurs in the Cytoplasm of Liver and Muscle

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8
Q

What is the rate-limiting enzyme in FA synthesis?

A

Acetyl-CoA Carboxylase (ACC)

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9
Q

What is the rate-limiting enzyme in Beta Oxidation?

A

Carnitine Acyltransferase -1 (CAT-1)

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10
Q

What is the rate-limiting enzyme in Cholesterol syntesis?

A

HMG CoA Reductase

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11
Q

What is the rate-limiting enzyme in Ketogenesis?

A

HMG CoA Synthase

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12
Q

What is the rate-limiting enzyme in Purine synthesis?

A

Glutamine - PRPP

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13
Q

What is the rate-limiting enzyme in Pyrimidine synthesis?

A

Carbamoyl Phosphate Synthetase-2 (CPS-2)

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14
Q

What is the rate-limiting enzyme in TCA Cycle?

A

Isocitrate dehydrogenase

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15
Q

What is the rate-limiting enzyme in Urea cycle?

A

Carbamoyl Phosphate Synthetase-1 (CPS-1)

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16
Q

What is the rate-limiting enzyme in Heme synthesis?

A

Delta-ALA Synthase

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17
Q

What are the catabolic pathways that create energy? (Occur in the Mitochondria)

A 
Acetyl-CoA production, 
Beta-oxidation, 
citric acid cycle, 
Ketogenesis, 
Oxidative phosphorylation
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18
Q

What are the anabolic pathways that store energy? (occur in the cytoplasm)

A

Fatty acid synthesis,
Glycolysis,
HMP shunt,
translation

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19
Q

What are the anabolic & catabolic pathways? (Occurs in both Cytoplasm and Mitochondria)

A

“HUG”
Heme synthesis
Urea cycle
Gluconeogenesis

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20
Q

What does an Isomerase do?

A

Creates and isomer

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21
Q

What does an epimerase do?

A

Creates an epimer, which differs around one chiral carbon

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22
Q

What does a mutase do?

A

Relocates a functional group within a moleculre (intrachain)

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23
Q

What does a Transferase do?

A

Relocates a functional group from one molecule to another

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24
Q

What does a Kinase do?

A

Adds Phosphate group to substrate using ATP

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25
Q

What does a Phosphorylase do?

A

Adds inorganic Phosphate to substrate, No ATP used

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26
Q

What does a Carboxylase do?

A

Transfers CO2 groups with the help of Biotin

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27
Q

What does a Synthase do?

A

Consumes two substrates

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28
Q

What does a Synthetase do?

A

Consumes two substrates, uses ATP

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29
Q

What does a Phosphatase do?

A

Removes Phosphate from substrate

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30
Q

What does a Hydroxylase do?

A

Adds-OH group onto substrate

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31
Q

What does a Lyase do?

A

Cuts C-C bonds w/ ATP

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32
Q

What does a Dehydrogenase do?

A

Catalyzes Oxidation-Reduction reactions (gaining or losing an electron)

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33
Q

What does a Thio do?

A

Breaks S bonds

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34
Q

What is Diffusion?

A

From high to low concentration No ATP

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35
Q

What is Active Transport?

A

Goes against concentration gradient

Requires ATP

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36
Q

What is Zero-order kinetics?

A

Metabolism independent of concentration (rate of elimination is constant)

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37
Q

What is 1st-order kinetics?

A

Constant drug percentage metabolism over time depends on drug concentration (rate of elimination is directly proportional to drug concentration)

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38
Q

What is Efficacy?

A

Maximal effect drug can produce regardless of dose (lower w/ non-competitive antagonist)

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39
Q

What effects Efficacy?

A

Vmax

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40
Q

What is Potency?

A

Amount of drug needed to produce effect (lower w/ comp antagonist)

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41
Q

What affects Potency?

A

Km (EC50)

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42
Q

What is Kd?

A

Concentration of drug that binds 50% of receptors

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43
Q

What is EC50?

A

Concentration of drug that produces 50% of maximal response

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44
Q

What is Competitive Inhibition?

A

Fights for active site, no change in Vmax, potency decreases

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45
Q

What is Non-competitive Inhibition?

A

Binds a regulatory site, no change in Km, Efficacy decreases, decreased Vmax

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46
Q

What is the Peak level?

A

4 hours after dose (too high - decrease dose)

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47
Q

What is the Trough level?

A

2 hours before next dose (too high - give less often)

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48
Q

What is t1/2?

A

Half-life, the time it takes the body to use half of the drug ingested

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49
Q

What is von Gierke?

A

GLYCOGEN STORAGE DISEASE
G-6 Phosphatase deficiency
severe fasting Hypoglycemia
hepatomegaly/renomegaly (increased glycogen in liver and kidneys)
increased lactase and uric acid levels (=>Gout)

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50
Q

What is Pompe’s?

A 
GLYCOGEN STORAGE DISEASE
Lysosomal a-1, 4-glucosidase deficiency
Die early/young due to heart failure
"Pompe trashes the Pump" (heart, liver, muscle)
exercise intolerance
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51
Q

What is Cori’s?

A

GLYCOGEN STORAGE DISEASE
Debranching, a-1,6 glucosidase enzyme deficiency
short branches of glycogen

milder form than Von Gierke with normal lactate levels
limit-dextrin’s accumulate

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52
Q

What is Anderson’s?

A

Branching enzyme deficiency,

long chains of glycogen

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53
Q

What is McArdle’s?

A

GLYCOGEN STORAGE DISEASE
Myophosphorylase deficiency (skeletal muscle Glycogen Phosphorylase)
Increased glycogen in muscle, but muscle cannot break it down
=> painful muscle cramps,
=> myoglobinuria (red urine with strenuous exercise)
And arrhythmia from electrolyte abnormalities
**Second-wind phenomenon (b/c increased muscular blood flow, tired athlete will find renewed energy to exercise harder)

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54
Q

What is Essential Fructosuria?

A

Fructokinase deficiency

Excrete fructose in blood/urine (still have hexokinase)

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55
Q

What is Fructosemia?

A

“Fructose intolerance” (Aldolase B deficiency)
kidney and liver damage
occurs in infants after introduction of fruits, honey, and juices

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56
Q

What does a Galactokinase deficiency cause?

57
Q

What does Galactosemia cause?

A

Cataracts, mental retardation, liver damage

58
Q

What does the Citrate shuttle do?

A

FA transport out of the mitochondria

59
Q

What does the Carnitine shuttle do?

A

FA transport into the mitochondria

60
Q

What lysosomal diseases have a cherry-red macula?

A

Tay-sachs, Niemann-Pick

61
Q

What lysosomal diseases have a Gargoyle face?

A

Gaucher’s (autosomal recessive; glucocerebroside accumulates in white blood cells of organs-macrophages; characterized by bruising, fatigue, anemia and low blood platelet count, enlarged liver/spleen, all due to deficiency of glucocerebrosidase).
Hurler’s (autosomal recessive; coarsening facial features 3-6months of age-prominent frontal bones, skull elongated, flattened nasal bridge, stop growing by age 2, all due to buildup of glycosaminoglycans due to alpha-L-iduronidase deficiency)

62
Q

What is Tay-Sachs?

A

Hexosaminidase A deficiency
Hyperreflexia developmental delay
“onion skin” Lysosomes

63
Q

What is Sandhoff’s?

A

Hexosaminidase A/B deficiency

64
Q

What is Gaucher’s?

A

Glucocerebrosidase (Beta-Glucosidase) deficiency
wrinkled tissue Macrophage’s
Bone pain
pancytopenia

65
Q

What is Niemann-Pick?

A

Sphingomyelinase deficiency
Areflexia
Hepatosplenomegaly
foam cells (Lipid Laden macrophages)

66
Q

What is Fabry’s

A 
alpha-galactosidase A deficiency
attacks baby's kidneys and heart
XLR
accumulation of Ceramide
Trihexoside
67
Q

What is Krabbe’s?

A 
Galactocerebrosidase deficiency
peripheral neuropathy
optic atrophy
globoid bodies
accumulation of Galactocerebroside and Psychosine
68
Q

What is Metachromatic Leukodystrophy?

A

Arylsulfatase A defieciency
childhood multiple sclerosis with Ataxia/Dementia
accumulation of Cerebroside Sulfate

69
Q

What is Hunter’s?

A 
Iduronate Sulfatase deficiency
milder form
XLR
no corneal clouding
aggressive behavior
Accumulation of Heparin and Dermatan Sulfate
70
Q

What is Hurler’s?

A 
Alph-L-Iduronidase deficiency
worse form
Gargoylism
Corneal clouding
accumulation of Heparin and Dermatan Sulfate
71
Q

What Is Lesch-Nyhan?

A 
HGPRT deficiency
grout neuropathy
self-mutilation
Dystonia
XLR
Prange sand crystals in diaper
72
Q

What do white diaper crystals suggest?

A

Excess Orotic acid

73
Q

What does biotin donate methyl groups for?

A

Carboxylation

74
Q

What does THF donate methyl groups for?

A

Nucleotides

75
Q

What does SAM donated methyl groups for?

A

All other reactions

76
Q

What is the difference between Heterochromatin and Euchromatin?

A 
Hererochromatin = tightly coiled
darker
Transcriptionally inactive
Euchromatin = loose (10nm fibers)
lighter
77
Q

What are the Purines?

78
Q

What are the Pyrimidines?

79
Q

What is a silent mutation?

A

Changes leave the same amino acid

80
Q

What is a point mutation?

A

Changes one base

81
Q

What is a transition?

A

Changes one purine to another purine

82
Q

What is a transversion?

A

Changes one purine to a pyrimidine

83
Q

What is a frameshift mutation?

A

Insert or delete any number of bases, not divisible by 3, causes misreading of all downstream nucleotides

84
Q

What is a missense mutation?

A

Mistaken amino acid substitution (i.e. SS disease Valine for Glutamic Acid)

85
Q

What is a nonsense mutation?

A

Early stop codon

86
Q

What does a Southern blot detect?

A

DNA, “Snow, Drop”

87
Q

What does a Northern blot detect?

A

RNA, “sNow, dRop”

88
Q

What does a Western blot detect?

A

Protein, “snoW, droP”

89
Q

What does Southwestern blot detect?

A

DNA-binding Proteins (combines south and western blots)

90
Q

What are the essential amino acids?

A 
Phynlalanine, Valine, Threonine
(PVT)
Tryptophan, Isoleucine, Methionine
(TIM)
Histidine, Arginine, Leucine, Lysine
(HALL)
91
Q

What are the essential fatty acids?

A

Linolenic, Linoleic

92
Q

What are the acidic amino acids?

93
Q

What are the basic amino acids?

A

Lys, Arg, His

94
Q

What are the sulfur-containing amino acids?

95
Q

What are the branched amino acids?

A

“Can’t LIVe w/o them”, Leu, Ile, Val

96
Q

What are the aromatic amino acids?

A

Phe, Tyr, Trp

97
Q

What is the smallest amino acid?

98
Q

What are the ketogenic amino acids?

99
Q

What are the glycogenic & ketogenic amino acids?

A

“PITT” Phe, Iso, Thr, Trp

100
Q

What are the glycogenic amino acids?

A

His, Met, Val

101
Q

What amino acid turns yellow on Ninhydrin reaction?

102
Q

What does Trypsin cut?

103
Q

What does Chymotrypsin cut?

A

Phe, Tyr, Trp

104
Q

What does alpha one anti-trypsin do?

A

Inhibits Elastase

105
Q

What is PKU?

A 
No Phe -> Tyr (via decreased Tetrahydrobiopterin Cofactor or decreased Phe Hydroxylase)
NutraSweet (Aspartame) sensitivity
mental retardation
pale
blond hair
blue eyes
musty odor
106
Q

What is Albinism?

A

No Tyr -> Melanin (via Tyrosinase)

107
Q

What is Maple Syrup Urine disease?

A

Defective metabolism of branched aa (Leu, Iso, Val) -> aa leak out, urine smells like Maple syrup/ burnt sugar, defect of branched chain alpha Keto acid Dehydrogenase

108
Q

What is Homocystinuria?

A 
No Homocysteine -> Cysteine
(Cystathionine Synthase Deficiency)
or Homocysteine Methyltransferase
(Methionine Synthase) Deficiency or decreased Affinity of Cystathionine Synthase for Pyridoxal Phosphate
Increased Homocysteine in urin
Marfanoid Habitus
downward dislocation of lens
increased risk stroke/MI
109
Q

What is Cystinuria?

A

Defect of AA transporter in PCT and GIm prevents reabsorption of Cysteine, Ornithine, Lysine, Arginine (COLA)
Hexagonal (Cysteine) stones in urine (urinary Cyanide nitroprusside test is Dx)

110
Q

What is Pellagra?

A

Niacin (B3) deficiency

Dermatitis, Diarrhea, Dementia, Death

111
Q

What is Hartnup’s?

A

No tryptophan-cannot make niacin or seratonin
Presents like Pellagra
Genetic, not nutritional disorder

112
Q

What causes anterior leg bowing?

A

Neonatal syphilis

113
Q

What causes lateral leg bowing?

114
Q

What are the names of the B vitamins?

A 
"The Rich Never Lie about Panning Pyrite Filled Creeks"
Vit B1 = Thiamine
Vit B2 = Riboflavin
Vit B3 = Niacin
Vit B4 = Lipoic acid
Vit B5 = Pantothenic acid
Vit B6 = Pyridoxine
Vit B7 = Biotin
Vit B9 = Folate
Vit B12 = Cobalamin
115
Q

What does Vit A do?

A

Night vision, CSF production, PTH cofactor

antioxidants

116
Q

What does Vit B1 do?

A

Dehydrogenases
transketolase (PPP)
cofactors

117
Q

What does Vit B2 do?

A

FAD cofactor

118
Q

What does Vit B3 do?

A

NAD cofactor

119
Q

What does Vit B4 do?

A

Glycolysis, no known diseases

120
Q

What does Vit B5 do?

A

Part of Acetyl-CoA, no known diseases

121
Q

What does Vit B6 do?

A

Transaminase cofactor, myelin integrity

122
Q

What does Vit B7 do?

A

Carboxylation (Avidin in egg whites (raw) binds Biotin)

123
Q

What does Vit B9 do?

A

Nuclear division (Synthesis of Nitrogenous bases for RNA and DNA)

124
Q

What does Vit B12 do?

A

Cofactor for HMT & MMA

125
Q

What does Vit C do?

A

Collagen synthesis
antioxidant
helps absorb FE++, necessary for Dopamine & hydroxylase for NE

126
Q

What does Vit D do?

A

Mineralization of bones and teeth

127
Q

What Does Vit E do?

A

Antioxidant

acts as an tagonist to Vit K in high levels

128
Q

What does Vit K do?

A

Clotting

Synthesized by intestinal flora

129
Q

What does Ca2+ do?

A

Neuronal function, atrial depolarization, smooth muscle contractility

130
Q

What does Cu2+ do?

A

Collagen Synthesis

131
Q

What does Fe2+ do?

A

Hb function, electron transport

132
Q

What is Bronze pigmentation?

A

Fe deposit in skin

133
Q

What is Bronze cirrhosis?

A

Fe deposit in liver

134
Q

What is Bronze diabetes?

A

Fe deposit in pancreas

135
Q

What is Hemosiderosis?

A

Fe overload in bone marrow

136
Q

What is Hemochromatosis?

A

Fe deposit in organs

137
Q

What does Mg2+ do?

A

PTH and kinase cofactor

138
Q

What does Zn2+ do?

A

Taste buds, hair, sperm function

tsomkapQ (117 decks)

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