Chapter 4: Protein Structure and Function Flashcards

1
Q

When a carboxyl group of the amino acid on the left attaches to the amino group of another AA on the right what does it make?

A

peptide bond

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2
Q

What is the difference between small proteins and large proteins in gel electrophoresis?

A

small proteins migrate further
large proteins stay closer to the start site

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3
Q

How does acidosis affect our bodies?

A
  1. denatures proteins
  2. hyperkalemia
    3.Kussmaul breathing
    4.more GABA
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4
Q

Which is the only enzyme that cuts to the left?

A

carboxypeptidase

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5
Q

Which enzyme cuts to the right of LYS and ARG?

A

Trypsin (trippin in LA)

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6
Q

Which enzyme cuts to the right of PHE, TRP, TYR

A

chymotripsin

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7
Q

Which enzyme cuts to the right of GLY, ALA, SER

A

Elastase (GAS to “last”)

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8
Q

Which cuts to the right of MET?

A

CNBr

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9
Q

Which enzyme cuts tot he right of the amino terminal amino acid?

A

Aminopeptidase

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10
Q

Which enzyme can break disulfide bonds?

A

Mercaptoethanol

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11
Q

Name two acute phase reactants?

A

CRP and ESR

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12
Q

What is the best test to detect excess protein in the serum during infections?

A

PROCALCITONIN

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13
Q

Which cells make acute phase reactants?

A

T cells and macrophages

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14
Q

Non specific deposition of acute phase reactants?

A

Amyloidosis

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15
Q

Diagnosis of Acute Amyloidosis

A

apple green birefrigence on Congo red stain

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16
Q

Amyloid alpha in developing countries?

A

chronic infection

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17
Q

Amyloid alpha in developed countries?

A

RA,SLE,IBD

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18
Q

Alpha Beta amyloidosis are found in which groups?

A

Downs syndrome and Alzheimer pts

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19
Q

Which betalipoprotein is most common in Alzheimers?

A

Betalipoprotein E4

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20
Q

What are neurofibrillary tangles of Alzheimers?

A

oxidized proteins building up

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21
Q

What is the main amyloid in Alzheimers?

A

Amyloid Precursor protein (APP)

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22
Q

What vitamin can you give Alzheimer patients to prevent oxidation of the proteins?

A

Vitamin E

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23
Q

Which amyloid is associated with renal failure?

A

AB-2

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24
Q

Which amyloid is associated with MEN2?

A

AE and AF

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25
Q

Which amyloid is associated with Multiple Myeloma?

A

AL L is for light chains (Bence Jones proteins in urine)

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26
Q

Which amyloid is associated with aging?

A

transthyretin

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27
Q

When two or more proteins are interacting with each other it is called

A

cooperativity

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28
Q

When two or more enzymes are cooperating it is called

A

allosterism (the allosteric enzyme is the rate limiting enzyme because it controls everything)

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29
Q

When substrate concentration no longer affects Vmax, all the binding sites are filled up, this is called

A

zero order elimination

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30
Q

first order elimination

A

proportion or percent eliminated per hour

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31
Q

When something says eliminates a set amount every hour that is

A

zero order elimination

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32
Q

examples of drugs that are zero order

A

alcohol, aspirin, phenytoin

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33
Q

When half the enzyme’s active sites are occupied by drug that is called

A

Km Km=1/2Vmax

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34
Q

What is the main difference between competitive and noncompetitive inhibitors?

A

Competitive inhibitors bind to the same site, the Km increases and the Vmax stays the same; noncompetitive inhibitors bind to different (allosteric) sites, Vmax decreases and Km stays the same.

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35
Q

Name an organ where you will find the lowest Km for any substrate (highest affinity or potency)?

A

brain

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36
Q

Which hemoglobin has 2 alpha-2beta chains?

A

HbA (adult)

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37
Q

Which hemoglobin has 2 alpha-2 delta?

A

HbA 2 (2% normal)

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38
Q

Which hemoglobin has 2 alpha-2 gamma chains?

A

HbF (fetal)

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39
Q

Which hemoglobin doesn’t like to drop off it oxygen?

A

HbF

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40
Q

What does hydroxyurea do in sickle cell pts?

A

induces more HbF

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41
Q

What is right shift of the oxygen hemoglobin dissociation curve mean?

A

It means hemoglobin willing to give up oxygen because hypoxia, acidosis, high altitude, increase in 2,3 BPG, or increase in temperature

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42
Q

What does left shift mean?

A

Hemoglobin less willing to give up oxygen because increase in HbF, CO, or Met hemoglobin.

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43
Q

When you are exercising, you have created a right shift, and if you keep pushing the body to oxygenate, you will force HbF and myoglobin to desaturate and give their oxygen, this is called?

A

second wind

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44
Q

When CPK leaks out of a muscle it is called?

A

myositis

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45
Q

What are the top reasons for myositis?

A
  1. hypothyroidism 2. Cushings 3. drugs (statin, INH, rifampin, steroids) 4. infection i.e. taenia solium from raw pork
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46
Q

What is a reserve source of energy in muscle called?

A

myoglobin

47
Q

Will acidosis shift the curve to left or right?

A

right

48
Q

How can you tell an athlete is blood doping?

A

high hemocrit, low EPO

49
Q

What is the antibody for Dermatomyositis?

A

anti-Jo

50
Q

What is the antibody for polymyositis?

A

anti-Jo

51
Q

labs in polymyositis?

A

up CPK, up LDH, up aldolase, up AST and ALT

52
Q

Where can erythropoiesis take place before 1 year of age?

A

liver, spleen, flat bones

53
Q

If you lose bone marrow function after 1 year old, which organ can take over erythropoeisis?

A

spleen (massive splenomegaly)

54
Q

Does synthesis take place in cytoplasm or mitochondria?

A

cytoplasm

55
Q

Does catabolism (breaking down) take place in cytoplasm or mitochondria?

A

mitochondria

56
Q

What is the exception to sythesis in cytoplasm rule?

A

glycolysis, breaks down in cytoplasm because it must feed into other pathways

57
Q

5 pathways that occur in both cytoplasm and mitochondria:

A
  1. heme synthesis
  2. gluconeogenesis
  3. urea cycle
  4. fatty acid synthesis
  5. pyrimidine synthesis
58
Q

What is the rate limiting enzyme for heme sythesis?

A

D-ala sythase

59
Q

What is it called when iron on hemoglobin is in Fe3+ state?

A

methemoglobinemia

60
Q

What is the treatment for methemoglobinemia?

A

methylene blue

61
Q

What is the treatment for cyanide poisoning?

A

amylnitrite to change iron to Fe3+ and bind cyanide

62
Q

most common causes of methemoglobinemia?

A

1.infection (free radicals made by neutrophil NADPH oxidase)
2. sulfa drugs

63
Q

What is the treatment for cyanide poisoning?

A

amylnitrite to change iron to Fe3+ and bind cyanide; B12 to bind cyanide and excrete through kidneys, methylene blue to reduce iron back to Fe2+, transfuse with fluids

64
Q

MCC of microcytic hypochromic anemia

A

Iron deficiency (poor diet) in ages 0-20; in ages 20-40 IBD; in ages greater than 40 mucosal bleeding; administer iron with vitamin C to protect in GI and vitamin E to protect in blood

65
Q

How is the Serum Fe and saturated Fe and Ferritin (stored iron) in iron deficiency?

A

all low

66
Q

What is the most common IBD in middle aged pts?

A

CrohnsWhat

67
Q

What is the main difference between iron deficiency anemia and anemia of chronic disease?

A

Anemia of chronic disease the Ferritin is high because the iron is sequestered; iron deficiency the Ferritin is low because of low intake or high loss.

68
Q

Which lab shows lead poisoning?

A

FEP free erythrocyte protoporphyrins

69
Q

What is drug for moderate lead poisoning?

A

Succimer

70
Q

What is drug for severe lead poisoning?

A

EDTA, Dimercaprol (BAL) and/or penicillamine

71
Q

EDTA treatment leads to deficiency of these 5 minerals?

A

Ca, Fe, Pb, Cu, Mg (any metal with 2+)

72
Q

Enzyme deficiency in Porphyria Cutanea Tarda

A

uroporphyrinogen decarboxylase

73
Q

Tx for Porphyria Cutanea Tarda

A

stay away from direct sunlight, use red/blue light, need blood transfusions, vita D deficiency, phlebotomy if necessary, anti-malarials (low dose ( removes excess porphyrins from liver)

74
Q

Which enzyme is deficient in Acute Intermittent Porphyria (AIP)

A

uroporphyrinogen 1 synthase

75
Q

5 P’s of AIP

A

pain (abd), polyneuropathy, psychological abnormalities, pink urine, precipitated by drugs (alcohol, barbs, OCP)

76
Q

treatment for AIP

A

opiates for pain, IV normal saline, clucose to bilirubin conjugate to help excrete from liver faster, hematin to block d-ala-synthase to decrease synthesis of rings

77
Q

signs of opiate withdrawal

A

cramps, diarrhea, excessive urination and piloerection

78
Q

What is the genetic problem with sickle cell anemia?

A

glutamic acid (neg.charge and water soluble) into valine (neutral, fat-soluble) at position 6 of beta chain

79
Q

What is the risk for any disease in general population? with one factor? with two factors

A

1-3%; one factor, 10%; two factors, more than 20%

80
Q

In sickle cell patients, always check reticulocyte count, why?

A

Make sure bone marrow is working

81
Q

What happens to spleen of sickle cell patients?

A

self asplenectomy by age 6 due to infarcts

82
Q

What kind of organisms are sickle cell patients susceptible to?

A

encapsulated organism Some Killers Have Nice Capsules Salmonella, Strep Pneumo, Hib, N. Meningiditis, need pneumo vaccine before age 2

83
Q

What is it called when one alpha gene missing in hemoglobin?

A

alpha thalassemia minor

84
Q

What is it called when all four alpha genes are missing?

A

alpha thalassemia major (incompatible with life)

85
Q

What is it called when one Beta gene is missing?

A

Beta thalassemia minor

86
Q

What is it called when two beta genes are missing from hemoglobin?

A

beta thalassemia major; need transfusions, can make HbA2 and HbF; transfusion dependent after 6 months of age

87
Q

How much more will hemoglobin raise if given 1 unit of packed red blood cells + one unite of FFP+ 1 unit of plts?

A

1-2 grams (3-6HCT)

88
Q

What is TRALI (transfusion related acute lung injury) caused by?

A

release of cytokines, causes ARDS, treat with steroids

89
Q

What can you get from too many transfusions?

A

Iron overload; hemosiderosis; hemochromatosis, skin bronzing, restrictive cardiomyopathy and arthritic pain in joints

90
Q

DOC for iron overload

A

deferoxamine, or EDTA-BAL)

91
Q

4 types of collagen

A

SCAB
Type 1-Skin
Type 2- Connective tissue
Type 3-Arteries
Type 4-basement membrane

92
Q

What is collagen made of (3 AA, 1 vitamin, 1 mineral)

A

glycine, proline, lysine , Vita C and Cu

93
Q

What is the only protein completely modified in the RER?

A

collagen

94
Q

What is the only modification that can go on in the RER?

A

N-acetylation (adding acetly group onto N)

95
Q

What is the chaperone to the Golgi?

A

HSP-90

96
Q

If mannose 6 phosphate is added in the Golgi, where will the protein be directed to?

A

lysosome

97
Q

In which disease is there a mannose 6 phospate deficiency, proteins are excreted into plasma and lysosomes are empty?

A

I cell disease

98
Q

What is the chaperone from RER to mitochondria?

A

HSP-70

99
Q

Which disease involves collagen type 1 and shows multiple bone fractures from birth?

A

Osteogenesis imperfecta (OI)

100
Q

What does CREST stand for in CREST syndrome?

A

Calcinosis cutis (calcium deposits in skin)
Raynaud’s (finger turn blue then white, then red
Esophageal immotility
sclerodactyly
telangiectasias

101
Q

What is the antibody involved in CREST?

A

anti-centromere antibodies

102
Q

Which antibody is involved in scleroderma?

A

anti-smooth muscle antibodies; anti-SCL 70;

103
Q

Which antibody is involved in progressive systemic sclerosis?

A

anti-topoisomerase antibody

104
Q

What is the most sensitive antibody in Rheumatoid Arthritis?

A

Anti cyclic citrullinated peptide (CCP antibody)

105
Q

What is the treatment for moderate to severe arthritis?

A

methotrexate, within 90 days add TNF inhibitor (imatinib)

106
Q

What antibodies are seen in Sjorgen’s syndrome?

A

anti-Ro, anti-La, SSA, SSB antibodies

107
Q

4 bad associations of cardiolipin antibody:

A

stimulates intrinsic clotting system
blocks vWF
multiple spontaneous abortions
false positive VDRL

108
Q

antibodies of SLE

A

anti-Smith, anti dsDNA, anti cardiolipin

109
Q

Tx for SLE

A

mycophenalate; steroids

110
Q

drug induced lupus commonly caused by

A

HIPPPI
hydralazine
INH
procainamide
penicillamine
phenytoin
ethosuximide

111
Q

Lupus + RA

A

mixed connective tissue disease, ribonuclear protein antibody (anti-RNP)

112
Q

What are neutropenic, burn pt, CF pts, and diabetics afraid of?

A

staph Aureus and pseudomonas

113
Q

tx for staph Aureus

A

vancomycin

114
Q

Tx for pseudomonas

A

2 antibiotics; i.e. piperacillin/tazo; ceftazidine/avibactim; aminoglycoside+betalactam