specific proteins 2 Flashcards

1
Q

Binds thyroxine; transports vitamin A

A

Prealbumin

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2
Q

molecular weight of prealbumin

A

62,000 Da,

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3
Q

prealbumin is also called

A
thyroxine-binding prealbumin (TBPA)
or transthyretin (TTR)
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4
Q

for Oncotic pressure; amino acid reservoir;

carries small molecules

A

albumin

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5
Q

protease inhibitors

A

a-antitrypsin

α2-Macroglobulin

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6
Q

Binds hemoglobin

A

Haptoglobulin

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7
Q

for Lipid transport

A

β-Lipoprotein

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8
Q

Transports iron

A

Transferrin

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9
Q

Component of complement system

A

C3

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10
Q

Clot formation

A

Fibrinogen

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11
Q

for Surface immunity

A

Immunoglobulin A

Immunoglobulin D

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12
Q

Binds to mast cells; hypersensitivity reactions

A

Immunoglobulin E

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13
Q

Humoral immunity primary response

A

Immunoglobulin M

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14
Q

is a small protein of only 182 amino acids, and so it would be rapidly
removed from the circulation by filtration through the kidney if it were
not held in the plasma by the larger protein prealbumin

A

retinol-binding protein (RBP

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15
Q

Prealbumin is rich in

A

tryptophan

β-pleated sheet conformation

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16
Q

source of the β-fibrillar amyloid component in type

I familial amyloidotic polyneuropathy

A

Prealbumin

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17
Q

halflife of prealbumin

A

roughly 2 days

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18
Q

detected from patients who have had heparin therapy

A

prealbumin

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19
Q

has major clinical utility as a marker for
nutritional status;
considered to be a better early
indicator of change in nutritional status than other commonly used
markers,

A

prealbumin

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20
Q

is used only as a landmark to

confirm that the specimen was likely CSF.

A

presence of a distinct band of prealbumin

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21
Q

True prealbumin is generally
below the level of detection by serum electrophoresis; instead it is best
quantified by

A

immunologic measurements such as nephelometry

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22
Q

crosses more easily into the

CSF than do the other serum proteins.

A

prealbumin

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23
Q

is usually

requested for detection of oligoclonal bands of immunoglobulin

A

Electrophoresis of CSF

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24
Q

what is present in prealbumin– apolipoprotein or beta-lipoprotein?

A

apolipoprotein

-B-lipoprotein is not present

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25
Q

The single most abundant protein in normal plasma is

A

albumin

2/3 of total plasma

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26
Q

loss of albumin results to

A

peripheral edema

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27
Q

the congenital absence of albumin (analbuminemia) generally does not lead
to such problems like edema because|___

A

lifelong compensatory mechanisms

that control hydrostatic pressures

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28
Q

The primary sequence of albumin contains

A

three major

regions with three peptide loops each

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29
Q

has regions of homology with serum albumin

A

α-fetoprotein

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30
Q

most common allotype of albumin

A

albumin A

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31
Q

how many amino acids are in albumin? and how is albumin arranged

A

585 amino acids arranged in

nine loops held together by the disulfide bonds between cysteine residues

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32
Q

percentage of albumin circulating in normal persons becomes glycosylated
nonenzymatically

A

8%

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33
Q

The half-life of circulating albumin is

A

about 17 days

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34
Q

other term for glycosylated albumin

A

fructosamine

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35
Q

Diabetic patients on hemodialysis can be

monitored with

A

glycosylated hemoglobin or glycosylated albumin

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36
Q

serves as a mobile repository of amino acids for incorporation into other
proteins

A

albumin

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37
Q

The primary structure of albumin

consists of

A

35 cysteine residues, of which 34 form intramolecular disulfide
bonds and one remains free

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38
Q

can be very useful for assessing diabetic control

in patients with hemolytic anemias

A

Measurement of glycosylated albumin

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39
Q

general transport or carrier protein

A

albumin

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40
Q

Analysis of newly synthesized albumin from intracellular sites has
revealed the existence of

A

precursor proalbumin

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41
Q

measures albumin and total protein

A

comprehensive metabolic panel
hepatic function
panel

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42
Q

measures albumin only

A

renal function panel

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43
Q

sensitive but
nonspecific reduction of albumin in so many different conditions has led
to its being termed a

A

“negative acute phase reactant”

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44
Q

these ions are
bound to albumin, and so decreases in albumin are directly responsible for
depression of their concentrations, too.

A

calcium and magnesium

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45
Q

compensatory mechanism showed by patients with cirrhosis

A

major polyclonal increase of immunoglobulins

in the γ-fraction

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46
Q

compensatory mechanism showed by patients with nephrotic syndrome

A

shows high levels

of α2-macroglobulin

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47
Q

Progression of diabetic nephropathy can be assessed by

A

quantitative measurement of albuminuria

48
Q

considered a standard of care for management of diabetes mellitus and the
early detection of diabetic complications.

A

Immunologic measurement of microalbumin

49
Q

The major component of the α1-globulins is

A

protease inhibitor α1-

antitrypsin (AAT)

50
Q

AAT is coded for by ___

A

SERPINA1 on chromosome 14.

51
Q

are suspected to have increased risk of lung or liver disease

A

SS and

SZ phenotypes

52
Q

most important protease inhibitor in plasma?

A

α1-

antitrypsin (AAT)

53
Q

Therapy for pulmonary emphysema secondary to AAT deficiency has
been greatly advanced by

A

intravenous replacement of AAT, using concentrates

or recombinant protein

54
Q

largest major nonimmunoglobulin protein

in plasma

A

α2-Macroglobulin (AMG)

55
Q

molecular weight of α2-Macroglobulin (AMG)

A

725,000 Da

56
Q

AMG

inactivates proteases by

A

complexing with them and forming covalent bonds

to them.

57
Q

at least 4 molecules of AMG differ in what components?

this can be separated by?

A

sialic acid
mannose
galactose

-isoelectric focusing

58
Q

The other major protein migrating in the α2-region which
has the function of combining with hemoglobin released by lysis of red
cells to preserve body iron and protein stores.

A

haptoglobin

59
Q

halflife of haptoglobin

A

roughly 4 days

60
Q

Hemoglobin–
haptoglobin complexes are removed from the circulation within minutes
by

A

reticuloendothelial system

61
Q

different

forms of haptoglobin can be distinguished by

A

high-resolution electrophoresis

62
Q

gives rise to a single

molecular species of molecular weight 100,000.

A

Normal haptoglobin (Type 1-1)

63
Q

Haptoglobin can be quantitated in terms of…

A

hemoglobin-binding

capacity or by immunologic means, especially nephelometry.

64
Q

low serum haptoglobin suggests

A

hemoglobinuria
(hemolysis)

Lactate dehydrogenase (LD) isoenzyme 1 (ASSOCIATED ENZYME)

65
Q

high serum haptoglobin suggests

A

myoglobinuria.

LD5 and creatine kinase (associated enzyme)

66
Q

The major β-globulin is

A

transferrin

67
Q

how many amino acids does transferrin have?

A

687 amino acids

68
Q

transferrin has a molecular weight of

A

79,550 Da

69
Q

In normal serum, transferrin ranges in concentration from

A

200–400 mg/

dL

70
Q

Current strategies

to screen for hemochromatosis include

A

measurement of serum iron

and serum transferrin

71
Q

is a rare disorder

characterized by microcytic anemia and iron overload

A

Congenital deficiency of transferrin (atransferrinemia)

72
Q

Persons who
engage in heavy alcohol consumption demonstrate abnormal ____

-generally considered to be a highly specific marker for chronic
alcohol abuse

A
carbohydratedeficient
transferrin (CDT) in their serum
73
Q

Depression of C3 occurs

in

A

autoimmune disorders

74
Q

is cleaved to form C3c, which migrates anodally to native

C3 as a band nondistinct from other β-globulins

A

C3

75
Q

a
convenient marker for assessing disease activity in rheumatic disorders
such as lupus erythematosus and rheumatoid arthritis.

A

C3 (and also C4)

76
Q

C3 and C4 are now easily

quantitated by

A

nephelometry

77
Q

how much fibrinogen does plasma cell contain?

A

100–400 mg/dL

78
Q

molecular weight of fibrinogen

A

340,000 Da

79
Q

no
fibrinogen is synthesized, results in a hemorrhagic disorder that paradoxically
is not as severe as the hemophilias

A

Congenital afibrinogenemia

80
Q

Migrating with the α2-globulins is a copper-binding protein

A

Ceruloplasmin

81
Q

where is ceruloplasmin synthesized?

A

liver

82
Q

what is ceruloplasmin’s molecular weight

A

132,000 Da
- consists of a single
polypeptide chain.

83
Q

exhibits ferroxidase

activity that is important in iron metabolism

A

Ceruloplasmin

84
Q

normal serum level of ceruloplasmin in adults

A

20–40 mg/dL

85
Q

each molecule of ceruloplasmin can bind how many atoms of copper?

A

6

86
Q

yellow color in ceruloplasmin is contributed by?

A

chromogens of plasma

87
Q

Iron is oxidized from ferrous to ferric ions by

A

ceruloplasmin

88
Q

results from disordered
copper metabolism, in which hepatic excretion of copper into the bile is impaired, leading to toxic deposition of copper in tissues

A

Wilson’s disease (hepatolenticular degeneration

89
Q

treatment of wilson’s disease

A

long-term
chelation with penicillamine

in severe cases, liver transplantation

90
Q

Diagnosis of Wilson’s disease is based on physical findings like

A

liver disease,
neurologic signs
Kayser-Fleischer ring in the cornea

91
Q

The oxidase activity of ceruloplasmin can be

used in a

A

colorimetric assay, with p-phenylenediamine as substrate, for
quantitation.

92
Q

Mutations in the ceruloplasmin gene leading to aceruloplasminemia result
in

A

iron overload

93
Q

(vitamin

D–binding protein [DBP])

A

Gc-Globulin

94
Q

molecular weight of gc globulin

A

51,000 Da

95
Q

Gc-globulin has two autosomal codominant alleles expressed as
three phenotypes

A

1-1, 2-2, and 1-2

96
Q

As a minor component of plasma proteins, Gc-globulin can be

quantitated by

A

radioimmunoassay
radioimmunodiffusion
rocket
immunoelectrophoresis

97
Q

binds heme released by degradation

of hemoglobin

A

Hemopexin

98
Q

This protein, also known as orosomucoid, has a very high carbohydrate
content, which minimizes its visualization by standard protein stains

A

α1-Acid Glycoprotein

99
Q

molecular weight of α1-Acid Glycoprotein

A

44,000 Da

100
Q

halflife of α1-Acid Glycoprotein

A

about 5 days

101
Q

normal serum level of α1-Acid Glycoprotein

A

40–105 mg/dL,

102
Q

transport drugs like lidocaine and tyrosine kinase inhibitor STI571

A

α1-Acid Glycoprotein

103
Q

This serum constituent was discovered by mixing the serum of patients
who had recovered from pneumococcal infections, with C-polysaccharide
of that bacterium.

A

C-Reactive Protein

104
Q

it appears to serve as a general scavenger molecule

A

C-Reactive Protein

105
Q

The normal serum concentrations of C-Reactive Protein

A

100 ng/
mL at birth
170 ng/mL in children
470–1340 ng/mL in adults.

106
Q

Immunologic methods used to precipitate C-reactive proteins

A

nephelometry, precipitations, RIA, and enzyme immunoassay

107
Q

highly specific for

neutralizing chymotrypsin

A

α1-antichymotrypsin (AAC)

108
Q

complexes with prostate-specific

antigen, measured as the bound form of PSA by immunoassay

A

α1-antichymotrypsin (AAC)

109
Q

Its role in disease states is probably similar to that of
the major protease inhibitors in preventing autodigestion of tissues by
endogenous cellular enzymes

A

inter-α-trypsin inhibitor (IATI)

110
Q

it plays a role in neutralizing thrombin

A

antithrombin III (AT3)

111
Q

provide the bulk of plasmin-neutralizing

activity in serum

A

AAT, AMG, and AT3

112
Q

acts to prevent activation of plasminogen, thereby blocking fibrinolysis
at an early step.

A

PAI-1

113
Q

inactivates activated coagulation factors V and VIII.

A

Protein C

114
Q

is capable of inhibiting activated complement
components C1r and C1s plus some other coagulation and fibrinolytic
factors.

A

The C1 esterase inhibitor

115
Q

show decreases in all fractions, but the most dramatic reduction is
often seen in albumin compared with its normally high value as the most
abundant serum protein

A

Patterns of hypoproteinemia due to malnutrition or gross loss of
protein