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Clinical Chemistry > AMINO ACIDS AND PROTEINS > Flashcards

AMINO ACIDS AND PROTEINS Flashcards

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1
Q

building blocks of proteins

A

amino acids

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2
Q

amino acids provide ____ of the total body energy requirement

A

12-20%

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3
Q

amino acids contain at least one ____ and ____ group

A

N-terminal end amino group; C-terminal end carboxylic group

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4
Q

what links amino acids together to form a polypetide

A

peptide bonds

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5
Q

what differentiates amino acids from one another

A

chemical composition of their R group

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6
Q

these cannot be synthesized by the body and must be obtained from the diet

A

essential amino acids

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7
Q

example of proteolytic enzymes that cleave dietary proteins into constituent amino acids

A

pepsin and trpysin

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8
Q

give 10 essential amino acids

A

histidine, isoleucine, leucine, lysine, methionine, phenylalanine, threonine, tryptophan, valine, arginine

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9
Q

it is a class of inherited errors of metabolism in which there is an enzyme defect that inhibits the body’s ability to metabolize certain amino acids

A

aminoacidopathies

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10
Q

what is the enzyme deficient in phenylketonuria

A

phenylalanine hyroxylase (converts phenyalanine to tyrosine)

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11
Q

odor of urine in phenylketonuria

A

mousy

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12
Q

deficient enzymes in tyrosinemia

A

type I: fumarylacetoacetate hydrolase
type 2: tyrosine aminotransferase
type 3: 4-hydroxyphenylpyruvate dioxygenase

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13
Q

deficient enzymes in alkaptonuria

A

homogentisate oxidase

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14
Q

deficient enzymes in MSUD

A

branched-a ketoacid decarboxylase

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15
Q

deficient enzymes in isovaleric acidemia

A

isovaleryl coA- dehydrogenase

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16
Q

deficient enzyme in homocystinuria

A

cystathione B-synthase

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17
Q

deficient enzyme in cystinuria

A

none; it only has a defect in amino acid transport

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18
Q

what AA accumulate in MSUD

A

leucine, isoleucine, valine

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19
Q

what AA are defectively reabsorbed in cystinuria

A

lysine, ornithine, cysteine, arginine

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20
Q

Why are proteins amhoteric/ampholytic

A

because they can be acidic or alkaline depending on the body ph

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21
Q

at ph <3.5 proteins behave as

A

cation

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22
Q

at ph >3.5 proteins behave as

A

anion

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23
Q

what is isoelectric point

A

it is the ph at which proteins do not have a net charge (zwitterions)

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24
Q

what comprises proteins

A

carbon, hydrogen, oxygen, nitrogen, sulfur

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25
Q

what percentage of nitrogen do proteins have

A

16%

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26
Q

Analogy:
Intracellular proteins: ____
_____: ribosomes attached in the rer

A

free ribosomes

extracellular

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27
Q

which organ synthesizes proteins

A

liver

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28
Q

severe or chronic liver diseases cause a ___ in plasma protein concentration leading to ____

A

decrease; hypoproteinemia

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29
Q

proteins comprises ___ of the cell’s dry weight

A

50-70%

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30
Q

what differentiates proteins from lipids and carbohydrates in terms of storage

A

proteins do not have a designated storage depot in the body

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31
Q

negative nitrogen balance: catabolism ___ anabolism

positive: anabolism ___catabolism

A

> ; >

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32
Q

conditions where a negative nitrogen balance is seen

A

excessive tissue destruction such as burns, wasting disease, high fever, starvation

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33
Q

conditions where a positive nitrogen balance is seen

A

growth, pregnancy, repair processes

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34
Q

protein is a transport carrier for susbtances such as

A

bilirubin, heme, hemoglobin, iron, copper, drugs, hormones

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35
Q

it is the most flexible transport protein

A

albumin

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36
Q

it is the consequence of low osmotic pressure

A

edema

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37
Q

how many g of albumin is excreted in the urine per day when there is nephrotic syndrome

A

3-4 grams

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38
Q

it is the linear sequence of amino acids and determines the identity of the protein

A

primary structure

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39
Q

it involves the winding of the polypeptide chain

A

secondary structure

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40
Q

what are the common secondary structures

A

alpha-helix,beta pleated, bend conformation

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41
Q

it is the actual 3D conformation of the protein

A

tertiary structure

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42
Q

what protein structure is affected by denaturation

A

secondary, tertiary and quaternary

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43
Q

factors causing denaturation

A

heat, strong acid/alkali, enzymatic reactions, exposure to urea and other subs, exposure to UV

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44
Q

transport proteins: (3)

A

apotrasferrin, apoceruloplasmin, albumin

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45
Q

structural proteins (3)

A

collagen, elastin, keratin

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46
Q

what are simple proteins

A

contain a peptide chain which on hydrolysis yields only amino acids

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47
Q

example of fibrous proteins

A

fibrinogen, troponin, collagen

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48
Q

example of globular proteins

A

globin enzyme, peptide hormone, albumin

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49
Q

what are conjugated proteins

A

proteins composed of an apoprotein and a non protein moiety (prosthetic group)

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50
Q 
give examples of the ff: 
metalloproteins
lipoproteins
glycoprotein 
mucoprotein
nucleoprotein
A

metalloproteins: ferritin, flavoprotein, hemoglobin, ceruloplasmin
lipoprotein: vldl ldl hdl chylomicrons
glycoprotein: haptoglobin, and alpha-1 antitrypsin
mucoprotein: mucin
nucleoprotein: chromatin

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51
Q

these are the most frequently analyzed of all the proteins

A

plasma proteins

52
Q

plasma proteins are divided into:

A

albumin and globulin

53
Q

the blood panel for plasma protein analysis consists of

A

total protein
albumin
globulin
A:G ratio

54
Q

this is a sensitive marker of nutritional status

A

pre albumin

55
Q

pre albumin is the transport protein for:

A

T4 and retinol

56
Q

why is pre albumin used to confirm is sample is really CSF

A

pre albumin crosses the CSF more easily compared to other proteins

57
Q

albumin comprises __% of the plasma protein mass

A

50%

58
Q

albumin transports:

A

bilirubin, fatty acids, calcium, magnesium, drugs, Salicyclic acid, dyes

59
Q

albumin is responsbile for 80% of _____ of the ___

A

colloid osmotic pressure; intravascular fluid

60
Q

is albumin a neg or pos APR

A

neg

61
Q

lowest plasma levels of albumin is seen in

A

nephrotic syndrome

62
Q

conditions where hypoalbuminemia is seen

A

malnutrition and malabsorption
liver dx
protein losing enteropathy or GI los
renal dx (nephrotic syndrome or glomerulonephritis)
acute dx states
mutation resulting from an autosomal recessive trait causing analbuminemia and bisalbuminemia
hemodilution, increased capillary permeability, and sepsis

63
Q

hyperalbuminemia is seen in what conditions

A

dehydration

excessive albumin infusion

64
Q

globulins are group of proteins consisting of

A

alpha 1, alpha 2, beta, and gamma fractions

65
Q

what does A1 antitrypsin inhibit?

A

it inhibits neutrophil elastase which are released by WBC to fight infection

66
Q

it is the major inhibitor of protease activity (preventing self destruction of tissues)

A

A1 antitrypsin

67
Q

A1 antitrypsin is deficient in conditions such as

A

emphysematous pulmonary disease and juvenile hepatic cirrhosis

68
Q

where is A1 fetoprotein synthesized

A

it is synthesized initially in the fetal yolk sac and then by the fetal parenchymal cells of the liver

69
Q

when does the concentration of AFP peaks in the fetus

A

13th week of gestation

70
Q

Physiologic increase of AFP: ___
Pathologic increase of AFP: ____
Pathologic decrease of AFP: _____

A

pregnant women with twins
neural tube defects (spina bifida and anencephaly), low folate
down syndrome and trisomy 18

71
Q

QUAD marker

A

high HCG and inhibin A and low maternal serum AFP and urinary estriol

72
Q

AFP is used as a tumor marker for ___

A

hepatic and gonadal cancer

73
Q

A1 - acid glycoprotein (orosomucoid) contains the highest percentage of __ and ___

A

CHO and sialic acid

74
Q

why is orosomucoid negatively charged even in acid solution

A

because it has a low isoelectric point 2.7 and due to high sialic acid content

75
Q

orosomucoid provides a useful diagnostic tool for ___

A

neonates with bacterial infecitons

76
Q

A1 acid glycoprotein has high affinity to ___ and binds ___

A

progesterone ; quinidine

77
Q

A1- antichymotrypsin binds and inactivates ____

A

prostate specific antigen

78
Q

A1- antichymotrypsin is an integral component of ___ found in Alzheimer’s dx

A

amyloid plaques

79
Q

what does A1-antichymotrypsin inhibits

A

serine proteases like; cathepsin C, pancreatic elastase, chymase and chymotrypsin

80
Q

it transports lipids and is the major component of hdl

A

A1- lipoprotein

81
Q

this inhibits serine proteases

A

inter-alpha-trypsin inhibitor

82
Q

function of group specific globulin

A

it transports vitamin D and binds actin

83
Q

what are the A1 globulins

A

haptoglobin, a2-macrogloblulin, apoceruloplasmin

84
Q

what is the function of haptoglobin

A

it binds the free hemoglobin by its alpha chain

85
Q

haptoglobin is decreased in ___

A

intravascular hemolysis and hemoglobinuria

86
Q

it is the largest major nonimmunoglobulin protein in plasma

A

a2- macroglobulin

87
Q

where is a2- macroglobulin primarily found

A

intravascular space

88
Q

in what condition does the concentration of a2- macroglobulin increases 10x

A

nephrosis

89
Q

it is a copper binding alpha-2 glycoprotein that has enzymatic activities

A

apoceruloplasmin

90
Q

where is apoceruloplasmin synthesized

A

liver (6-8 atoms attached forming ceruloplasmin)

91
Q

what color does it impart to proteins

A

blue

92
Q

apoceruloplasmin is decreased in what conditions

A

Wilson’s dx, menke’s kinky hair syndrome

93
Q

copper deposits in the cornea

A

kayser fleischer rings

94
Q

what are the B- globulins

A

Pre-b lipoprotein; transferrin; hemopexin; B-lipoprotein; B2- microglobulin; complement, CRP

95
Q

Analogy:
Pre-b lipoprotein: ___
______: LDL

A

VLDL trigylcerides

b-lipoprotein

96
Q

Transferrin comprises ___ of the beta globulin fraciton

A

90%

97
Q

how many molecules of ferric ion does transferrin transport

A

2

98
Q

level of transferrin in IDA

A

normal or increased (paraprotein)

99
Q

what does hemopexin do

A

it binds the free heme and evaluates early hemolysis

100
Q

B2-microglobulin is a light chain component of MHC class __ which are found in ___

A

II; nucleated cells

101
Q

B2 microglobulin is recognized by ___

A

Cd8 or cytotoxic cells

102
Q

functions of complement

A

cell lysis; opsonization; anaphylaxis; inflammation

103
Q

which complement is present in high concentration in the plasma

A

c3

104
Q

CRP is named after its ability to bind with the ___ of ___

A

C-polysaccaride; pneumococci

105
Q

it is used as an early warning test to persons at risk of coronary artery disease

A

hsCRP

106
Q

it is the most abundant of the coagulation factors

A

fibrinogen (factor 1`)

107
Q

effect of high levels of fibrinogen in the plasma

A

it may elevate the ESR which favors rouleaux formation which sediments faster

108
Q

fibrinogen is found between __ and ___ globulin

A

beta and gamma

109
Q

antibodies in the neonatal serum are of ____ origin

A

maternal

110
Q

myoglobin is a primary-oxygen carrying protein found in ___ and ___

A

striated skeletal muscle and cardiac muscle

111
Q

give the onset, peak, and normalize of myoglobin

A

2-3 hrs; 8-12 hrs; 18-30 hrs

112
Q

myoglobin is not cardiospecific because it is also elevated in ___ and ___

A

progressive muscular dystrophy and crushing injury

113
Q

which troponins are considered as gold standard tess for acute coronary syndrome

A

T and I

114
Q

which troponin is present in both cardiac and skeletal muscle; binds calcium to regulate muscle contraction

A

C

115
Q

Analogy:
Troponin T: ____ ( onset: ___ peaks: ____ normalize: ___
____: myocardium

A 
heart muscle (3-4; 10-24; 7 days 
Troponin I
116
Q

it is a potent vasodilator that promotes natriuresis

A

natriuretic peptide

117
Q

fibronectin plays a role in ___, ___, ___, ____

A

cell adhesion, tissue differentiation, growth and wound healing

118
Q

B-trace protein is established as an accurate marker of ___

A

CSF leakage

119
Q

CTXs is a biochemical marker of ____

A

bone resorption

120
Q

Cystatin C is an endogenous marker of ___

A

GFR

121
Q

test used for differential diagnosis of Alzheimers dx

A

Amyloid b-42 and Tau test

122
Q

test indicating increased likelihood of alzheimer’s dx

A

low AB42 and high Tau test

123
Q

microalbuminuria is increased in ___, ____,___,___

A

fever, infection, hypertension, diabetic nephropathy

124
Q

it is the presence in CSF of two or more IgG bands in the gamma region

A

CSF oligoclonal banding

125
Q

other disorders with two or more bands in the CSF: (4)

A

neoplastic d/o. neurosyphilis, encephalitis, and guillan barre syndrome

126
Q

supporting medium and stain for CSF oligoclonal banding

A

agarose gel and coomasie brilliant blue stain

Clinical Chemistry (4 decks)

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