8) Proteins Flashcards

1
Q

basic protein formula

A

RCH(NH2)COOH

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2
Q

isoelectric point (pI)

A

the pH at which the amino acid or protein has no net charge, and the positive charges equal the negative charges

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3
Q

At a pH ——– than the pI, the protein carries a ——– charge

A

greater – negative
less – positive

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4
Q

peptide bond

A

Chemical bond between a carboxyl group of one molecule and an amino group of the other molecule, releasing water

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5
Q

Plasma ——— provided by the proteins tends to retain water in the vascular space

A

colloid osmotic pressure (COP)

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6
Q

3 main functions of plasma proteins

A
  • Maintenance of water distribution between cells and tissue.
  • Coagulation proteins are important in maintenance of hemostasis.
  • Transport vehicles to move various ligands to where they are needed or stored.
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7
Q

Rare inherited disease involving the homogentisic acid oxidase

Leads to a buildup of homogentisic acid in the tissues of the body

A

alkaptonuria

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8
Q

darkening of the tissues of the body because of the excess homogentisic acid

A

Ochronosis

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9
Q

Caused by the absence or very low levels of the branched-chain enzyme α-keto acid decarboxylase complex

A

MSUD

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10
Q

Results in the abnormal metabolism of three essential amino acids:

leucine, isoleucine, and valine

A

MSUD

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11
Q

Results in the inability to metabolize the essential amino acid phenylalanine

A

phenylketonuria

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12
Q

grouping of plasma proteins

A
  • Albumin
  • Globulin (α1 globulins, α2 globulins, ß1 globulins, λ globulins)
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13
Q

TP =

A

albumin + globulin

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14
Q

Main clinical significance of ———- is role as a sensitive marker of poor nutritional status such as protein-energy malnutrition (PEM).

A

prealbumin/transthyretin (TTR)

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15
Q

protein measured in CSF, not electrophoresis

A

prealbumin/TTR

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16
Q

albumin is approximiately —-% of serum protein

A

60

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17
Q

list the 16 plasma proteins
𝛼1 - 3
𝛼2 - 3
β - 5
𝛾 - 2

A

prealbumin
albumin

𝛼1-antitrypsin
𝛼1-acid glycoprotein
alpha-fetoprotein

haptoglobin
ceruloplasmin
𝛼2-macroglobulin

transferrin
hemopexin
β-lipoproteins
β2-microglobulin
C3

fibrinogen

C-reactive protein
Ig

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18
Q

albumin functions

A

Maintain plasma colloid osmotic pressure.

Bind and transport a wide variety of ligands.

Serve as an endogenous source of amino acids.

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19
Q

causes of hypoalbuminemia

A
  • increased catabolism (most common)
  • impaired synthesis
  • increased protein loss
  • analbuminemia
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20
Q

causes of hyperalbuminemia

A
  • Dehydration/decreased plasma volume
  • high protein diet
  • albumin infusion
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21
Q

A/G ratio

A

albumin/globulin

globulin = TP - albumin

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22
Q

RR for A/G ratio

A

0.8-2.2

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23
Q

Major α1–globulin, making up approximately 90% of α1–proteins.

A

α1–Antitrypsin (AAT)

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24
Q

one of the most common genetically lethal diseases in Caucasians (1:4000)

A

AAT deficiency

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25
Q

sx of AAT deficiency

A

emphysema with onset at 45 years of age or earlier
emphysema occurring in the absence of smoking

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26
Q

is the major glycoprotein increased during inflammation, an Acute Phase Reactant (APR).

A

α1–Acid Glycoprotein/orosomucoid
(AAG)

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27
Q

Principal fetoprotein (fetal albumin-like protein) in maternal serum

A

alpha-fetoprotein

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28
Q

Used to screen for the antenatal diagnosis of neural tube defects including spina bifida and anencephaly

A

AFP

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29
Q

—— is decreased in Down’s syndrome and Trisomy 18

A

AFP

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30
Q

increased levels found in 80% of patients with hepatocellular cancer, 50% of germ cell tumors (gonadal), and all children with hepatoblastoma

A

AFP

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31
Q

An acute phase reactant that binds free hemoglobin in plasma

A

haptoglobin (Hp)

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32
Q

Depletion is the most sensitive indicator of intravascular hemolysis, in transfusion reactions, and certain hemolytic anemias.

A

Hp

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33
Q

Principal copper (Cu)-containing protein in plasma containing 95% of the total serum copper

A

Ceruloplasmin (Cp)

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34
Q

Decreased in Wilson’s disease, Menkes disease, malnutrition, malabsorption, severe liver disease, nephritic syndrome

A

Cp

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35
Q

Rare autosomal recessive trait where Cp levels are reduced and the dialyzable Cu concentration is increased.

A

Wilson disease

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36
Q

copper chealators used to tx Wilson’s

A

penicillamine
trientine

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37
Q

effects of Wilson’s disease

A
  • Degenerative cirrhosis
  • Chronic active hepatitis
  • Renal tubular acidosis
  • Neurological damage (clumsiness, tremors)
  • Kayser-Fleischer rings, pigmented rings at the outer margins of the cornea and the sclera
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38
Q

In nephrotic syndrome, —— is characteristically increased above normal because it is retained while smaller proteins are excreted in the urine.

A

AMG

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39
Q

Major component of β-globulins
negative acute phase reactant

A

TRF

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40
Q

Principal plasma protein for transport of iron as Fe+3 — the ferric ion — to storage sites, where it is bound to apoferritin and stored as ferritin

A

TRF

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41
Q

Important in the differential diagnosis of anemias and monitoring the treatment of iron deficiency anemia

A

TRF

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42
Q

A commonly used indicator of iron overload—screen for hemochromatosis

A

TRF

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43
Q

When red blood cells are destroyed, it transports heme to the liver, where it is catabolized by the reticuloendothelial system.

A

hemopexin

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44
Q

Increased levels are found in pregnancy and in diabetes mellitus.

A

hemopexin

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45
Q

Comprises the common light chain of human leukocyte antigens (HLA) found in all nucleated cells

A

BMG

46
Q

Used for staging MM and for CLL survival

A

BMG

47
Q

Increased in renal failure, inflammation, and neoplasms—especially those associated with B-lymphocytes

A

BMG

48
Q

Collective term for 30 glycoproteins that participate in the immune reaction and serve as a link to the inflammatory response

A

C3

49
Q

Plasma — sharp peak between Beta and Gamma region on electrophoresis (artifact)

A

fibrinogen
absent in serum

50
Q

An acute phase reactant and a nonspecific indicator of bacterial vs viral infection, inflammation, and tissue injury or necrosis

A

CRP

51
Q

Reacts with proteins present in many bacteria, fungi, and protozoan parasites

A

CRP

52
Q

Levels rise dramatically following myocardial infarction, trauma, psychological or physical stress, infection, inflammation (e.g., rheumatic fever, rheumatoid arthritis), surgery, and various cancers.

A

CRP

53
Q

undetectable in healthy individuals

A

CRP

54
Q

Spike in Waldenstrom’s Macroglobulinemia

A

IgM

55
Q

can rarely see an —– spike in MM

A

IgA

56
Q

Increased in infections, liver disease,
connective tissue disorders

A

IgD

57
Q

—– proteins move toward the cathode (right)

A

gamma

58
Q

Transitory ——— elevations in pre-eclampsia of pregnancy and dehydration.

A

urinary protein

59
Q

indicator in protein pad of urine strip

A

tetrabromophenol blue

60
Q

urinary protein elevation causes

A

pregnancy
nephritic syndrome
acute and chronic glomerulonephritis
renal failure
lesions of the kidney
multiple myeloma

61
Q

RR for CSF protein

A

15-45 mg/dL

62
Q

Increased CSF protein levels indicate…

A

a broken blood/brain barrier

63
Q

Associated with a positive nitrogen balance

A

hyperproteinemia

(hemoconcentration or dehydration)

64
Q

characteristic electrophoresis pattern of MM

A

monoclonal spike in gamma region

65
Q

Caused by a negative nitrogen balance

A

hypoproteinemia

66
Q

Based on presence of peptide bonds found in all proteins.

A

biuret reagent

67
Q

When a solution of protein (serum or plasma) is treated with cupric (Cu+2) divalent ions in a moderately alkaline medium, a violet-colored chelate, which absorbs light at —– nm, is formed between the cupric ion and carbonyl oxygen and the amide nitrogen atoms of the peptide bond.

A

540

68
Q

Sodium potassium tartrate
Copper sulfate
Potassium iodide
NaOH

A

biuret reagent

69
Q

dye-binding method of protein analysis uses ———-, which is dissolved in an acidic solution, causing it to absorb at 465 nm

A

Coomassie Blue G-250

70
Q

RR for serum protein

A

6.0-8.3 g/dL

71
Q

A physiological decrease of approximately 0.5 g/dL protein occurs in …..

A

bedridden patients

72
Q

2 methods used for albumin

A

Bromocresol green
Bromocresol purple

73
Q

4 requirements for dye-binding albumin methods

A
  1. Specific binding of the dye to albumin in the presence of serum or plasma proteins
  2. High binding affinity between the dye and albumin
  3. Substantial shift in the absorption wavelength of the dye in the bound form
  4. Absorption maximum for the bound form at a wavelength distinct from those where bilirubin and hemoglobin, the main interfering chromogens, can interfere
74
Q

zwitterion

A

A molecule or ion having separate positively and negatively charged groups, with net zero charge

75
Q

relative protein %

A

relative % = absorbance of one band/absorbance of all bands

76
Q

high resolution electrophoresis

A

divides Beta band into 2

77
Q

Electrophoresis pattern shows a double albumin spike

A

bisalbuminemia

78
Q

a condition in which abnormal proteins (antibodies) of one type are found.

A

monoclonal gammopathy

79
Q

a condition in which results from an increased production of several different immunoglobulins.

A

polyclonal gammopathy

80
Q
  • Patient’s serum has an M-protein that measures <3 g/dL
  • Bone marrow that contains <10% of plasma cells
  • no CRAB
A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

81
Q
  • Patient’s serum has an M-protein that measures ≥3g/dL
  • Bone marrow that contains ≥10% of plasma cells
  • no CRAB
A

Smoldering Multiple Myeloma (SMM)

82
Q

Plasma cells light chains (pieces of antibodies) misfold and build up in organs, causing them to thicken and loose function

A

amyloid light-chain (AL) amyloidosis

associated with SMM

83
Q

Neoplastic proliferation of a clone of plasma cells

A

multiple myeloma

84
Q

b cell tumor

A

plasmacytoma

85
Q

hallmark of MM

A

lytic bone lesions

86
Q

free light chains in urine associated with MM

A

Bence Jones proteins

87
Q
A

Calcium (hypercalcemia)
Renal insufficiency
Anemia
Bone lesions/pain

88
Q

Overproduction by B lymphocytes, mainly in lymph nodes

A

Waldenstrom’s Macroglobulinemia (WM)

89
Q

Starts with an IgM MGUS

A

WM

90
Q

Enlarged lymph nodes
Rouleaux formation on peripheral smear
↑ ESR

A

WM

91
Q

Can precipitate and act as a cryoglobulin at low temperatures

Raynaud’s phenomenon
cold urticaria

A

WM

92
Q

Can lead to Hyperviscosity syndrome

A

WM

93
Q

adverse effects of hyperviscosity syndrome

A
  • Bleeding/vascular insufficiency
  • Visual impairments
  • Cardiac Insufficiency
  • Neurological side effects
  • Analyzer Issues
94
Q

Precipitated out upon heating to 50-60°C and redissolved upon further heating to 90-100°C

A

Bence-Jones proteins

95
Q

Motion of a liquid when a voltage is applied between the ends of an insulating tube that contains that liquid

A

electroosmosis

96
Q

methodologies used to ID protein levels and M spikes

A
  1. Electrophoresis (SPEP or UPEP)
  2. Immunotyping (IT)
  3. Immunofixation electrophoresis (IFE)
97
Q

principle of IT

A

Disappearance of the abnormality in the antiserum-treated pattern indicates the presence of a monoclonal protein.

98
Q

used to type any suspected monoclonal component previously detected by serum protein electrophoresis

A

Immunofixation Electrophoresis (IFE)

99
Q

IEF techniques are similar to electrophoresis except that the separating molecules migrate through a pH gradient.

A

isoelectric focusing
(not done anymore)

100
Q

—— is the most common cause of β-γ bridging.

A

cirrhosis

101
Q

Excessive loss of serum proteins into the GI tract

A

protein losing enteropathy (PLE)

102
Q

Results in hypoalbuminemia → poor surgical outcomes → risk of death

A

PLE

103
Q

Fecal excretion of alpha 1-antitrypsin

A

PLE

104
Q

↓ albumin
↑ 𝛼2
↑ 𝛾

A

chronic inflammation

105
Q

↓ albumin
↑ 𝛼2
variably ↑ β
↓ 𝛾

A

nephrotic syndrome

106
Q

↓ albumin
↑ 𝛾
briding

A

hepatic cirrhosis

107
Q

↓ 𝛾

A

hypogammaglobulinemia

108
Q

↓ albumin
↑ 𝛼2
↓ 𝛾

A

protein-losing enteropathy

109
Q

↓ albumin
↑ 𝛼1
↑ 𝛼2

A

acute inflammation

110
Q

↓ albumin
↑↑ 𝛾 spike

A

monoclonal gammopathy