Test 2: Proteins Flashcards

1
Q

What are two transport proteins that act as buffers?

A

albumin and hemoglobin

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2
Q

Transferrin can carry ____ iron and is usually only _____ saturated.

A

2, 1/3

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3
Q

Most proteins are ________ amino acids and synthesized in the liver.

A

200-300

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4
Q

What is the functional unit of the liver?

A

lobule

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5
Q

Immunoglobulins come from _______ cells.

Hb comes from __________.

A

plasma

bone marrow

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6
Q

There are ____ pyrrolines is heme with alternating double bonds.

A

4

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7
Q

What can be broken down to make immunoglobulins. Acts like storage

A

albumin

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8
Q

Where does AA absorption take place?

A

Ileum & Jejunum

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9
Q

Excess AA get degraded in the liver and is stored as…

A

“fat”

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10
Q

What partialy digests proteins?

A

stomach (HCL and pepsin)

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11
Q

[aa] blood: ______ mg/dl

A

35-65

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12
Q

Protein turnover rate is ______ g/day

A

125-220

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13
Q

Kidney Filters & reabsorption:

_________ g/dl of protein gets exreated in urine.

A

~7g/dl (150-300mg/24h)

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14
Q

1/3 of normally excreted protein is “ ___________ Protein”

A

Tamms Horsfall AKA Uromodulin (thick ascending loop of Henle)

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15
Q

What are the two protein catabolism pathways?

A

-Lysosomal pathway: Intra & extracellular proteins
-Cytosolic pathway: Intracellular proteins

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16
Q

Central catabolic reaction where amino can become keto and keto can become amino through pyridoxal phosphate.

A

Transamination/“Transaminase”

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17
Q

What are the exception of transamination?

A

Lysine, threonine, proline, hydroxyproline

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18
Q

Total protein = ________ + ________

A

Albumin, Globulin

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19
Q

When total protein goes down it is due to decreased ________.

A

albumin

-sign of hepatic damage (not in early stage)

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20
Q

When total protein goes up is it due to increased ___________.

A

Globulin.

-caused by inflammation

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21
Q

What is the A/G ratio used for?

A

to understand hepatic conditions more and with cirrhosis

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22
Q

Normal A/G ratio?

A

*** 1:1 - 1.5:1

   1.5:1 - 2.5:1
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23
Q

Total protein is approx…

A

6.5-8.3 g/dl

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24
Q

Total albumin is approx…

A

3.5-5.5 g/dl

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25
Q

Hemoconcentration & Hemodilution are __________ protein concentration changes in the blood.

A

relative

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26
Q

Hemoconcentration & Hemodilution there is no change in A/G ratio. The change is mainly due to what change?

A

plasma volume change

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27
Q

Low body H20 —–>

A

“Hemoconcentration”

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28
Q

Excess body H20 —>

A

“Hemodilution

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29
Q

What conditions effect water balance?

A

-Cushings
-Adison’s disease
-
-

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30
Q

Is Hypoproteinemia or Hyperproteinemia more common?

A

Hypoproteinemia

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31
Q

What can cause hyperproteinemia?

A

-Dehydration (most common)
-Increased γ-globulin
-myeloma
-Waldenstrom’s macroglobulinemia
-chronic infection

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32
Q

How does myeloma cause hyperproteinemia?

A

effects the kidneys (clogs glomerulus)
makes lots of immunoglobulins (but of bad quality)

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33
Q

known as non-hodkins lymphoma or lymphoplasmacytic (plasma cells)

A

Waldenstrom’s macroglobulinemia

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34
Q

What two renal conditions cause hypoproteinemia?

A

✴Nephrotic syndrome or Nephrosis

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35
Q

What conditions can cause hypoproteinemia?

A

1.Excretion in urine in renal disease
2.Leakage into the GI tract (“spruce”, malabsorption)
3.Loss of blood
4.Decreased Intake
5.Decreased synthesis
6.Increased protein catabolism
✴Burn, Trauma, increased energy demand
[pregnancy]

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36
Q

What are two Negative Acute Phase Reactants?

A

albumin and transferrin***

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37
Q

protein that decreases in acute phase

A

negative phase reactant

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38
Q

protein that increases in the acute phase.

A

-acute phase reactant
-acute phase protein

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39
Q

Acute phase reaction (or protein) is the response to…

A

the acute inflammation!

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40
Q

Acute phase reactants involves what functions?

A

defense or protective function

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41
Q

Why is C reactive protein (CRP) useful?

A

baseline levels are low, can dramatically increase

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42
Q

What are the immune acute phase reactants?

A

-Αmyloid P component (Serum)
-Mannose binding lectin (MBL)
-Complements

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43
Q

What are the coagulation acute phase reactants?

A

-Fibrinogen
-Prothrombin
-Factor VIII von willebrand factor
-Plasminogen activator inhibitor (PAI-1)

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44
Q

The only protein that increases increases in blood samples of nephrotic syndrome (cannot go through glomerulus)

A

α2 macroglobulin

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45
Q

What acute phase reactant down regulates inflammation?

A

α1-antitrypsin***

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46
Q

What are all the acute phase reactants?***

A

-CRP
-Αmyloid P component (Serum)
-Mannose binding lectin (MBL)
-Complements
-Fibrinogen
-Prothrombin
-Factor VIII von willebrand factor
-Plasminogen activator inhibitor (PAI-1)
-α2 macroglobulin
-Ferritin
-Ceruloplasmin
-Haptoglobin
-α1-antitrypsin
-α1-antichymotrypsin

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47
Q

What are the negative phase reactants?***

A

-Albumin
-Transferrin
-Transthyretin
-Retinol binding protein
-Antithrombin
-Transcortin (also called corticosteroid binding globulin, or serpin 6)

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48
Q

thyroid hormone carrier, prealbumin

A

Transthyretin

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49
Q

retinol (Vit A) carrier protein

A

Retinol binding protein

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50
Q

Transcortin is also called?

A

corticosteroid binding globulin, or serpin 6

it is a cholesterol carrier

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51
Q

What negative phase reactant is associated with protein and nutrition status?

A

pre albumin

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52
Q

What are the fat soluble vitamins?

A

Vitamins A, D, E, and K

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53
Q

What is the normal BUN/creatinine ratio?

A

10:1 - 20:1

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54
Q

If both BUN and creatinine is elevated, ratio will still appear normal…. What could this indicate?

A

renal disease

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55
Q

Pre-renal acute failure is usually due to altered ___________ function.

A

Cardiovascular

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56
Q

Serum BUN creatinine ratio grater than 20:1 is observed in patients with…

A

-pre-renal azotemia*** (accumulation of nitrogenous products in blood)
-gastrointestinal bleeding
-excessive protein intake

-post-renal azotemia

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57
Q

What is the difference between positive acute phase reactants and negative acute phase reactants?

A

*Negative acute phase reactants are downregulated, and their concentrations decrease during inflammation
(shunted to other protein needs, required in the current inflammatory process)

Positive acute phase reactants are upregulated, and their concentrations increase during inflammation.

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58
Q

When using an alkaline buffer with serum protein electrophoresis, where is the sample loaded?

A

the middel!

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59
Q

What is the order on serum protein electrophoresis (+ to -)

A

-pre-albumin
-albumin
-a1-anti trypsin, a1-acid glycoprotein
-a2-macroglobulin, haptoglobin
-hemopexin, transferrin, beta-lipoprotein, C3
-immunoglobulins

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60
Q

Biologically active form of thyroid hormone =

Inactive form =

A

T3 (3 iodine)

T4 (4 iodine)

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61
Q

[transthyretin (TTR) / thyroxine-binding prealbumin (TBPA)]

A

Prealbumin???

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62
Q

Serves as a transport protein for a small fraction of thyroid hormones,
esp. thyroxine

A

Prealbumin

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63
Q

Prealbumin also binds with ____________ protein to transport retinol (Vitamin A)

A

retinol-binding

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64
Q

True or false:

Pre-albumin is typically seen on SPE

A

False

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65
Q

What is a VERY sensitive marker of poor protein nutritional status?***

A

Prealbumin

66
Q

Decreased in hepatic damage (can be produced here), acute phase inflammatory response,
& tissue necrosis***

A

Prealbumin

67
Q

Prealbumin is increased in what patients?***

A

patients receiving steroids, & chronic renal failure (because it can go through the glomerulus)

68
Q

What is the half-life for albumin and prealbumin?

A

albumin - 20 days
prealbumin - 2 days (because it can go through the glomerulus)

69
Q

Normal range for albumin?

A

3.5-5.5 g/dl

MW: 66 kD (threshold of glomerulus)

70
Q

Albumin makes up ______% of total protein.

A

50-60

71
Q

What are the requirements for substance to be accurate for clearance testing.

A

-stable
-freely filtered
-no reabsorption
-no execration? (not an issue with inulin)

72
Q

What charge is albumin>\?

A

extremely negatively charged

73
Q

Albumin has a low pH of 4.0-5.8. In an alkaline buffer, moves rapidly towards the _______.

A

anode (+)

74
Q

What are the main functions of albumin?

A

-maintenance of colloid osmotic pressure of intravascular fluid, binding subs. in the blood “negativity”
-oncotic pressure
-source of AA (neg. acute phase reactant
-buffering capacity

75
Q

examples of substances in the blood that albumin binds to

A

Unconjugated bilirubin, salicylic acid, FA, Ca2+, Cortisol, & drugs

76
Q

What are the two types of causes for hypoalbuminemia?

A

-Decreased production
-Increased loss/use after synthesis

77
Q

What are the decreased production causes of hypoalbuminemia?

A

-Malnutrition (inadequate source of aa)
-Liver disease (not in early stage)

78
Q

What are the loss/use after synthesis causes of hypoalbuminemia?

A

-GI tract loss via intestinal leakage
-Loss in renal disease (not reabsorbed)
-Burns (leakage)
-Ascites
-Inflammation/neoplasm
-Pregnancy (used by baby)

79
Q

How is edema related to hypoalbuminemia?

A

decreased albumin causes the oncotic pressure to go down resulting in water leakage into tissues.

80
Q

Causes of hyperalbuminemia?

A

-Relative increase
-Usually due to dehydration
-Rarely seen due to other reasons

81
Q

-two peaks are seen for albumin
-Unusual molecular characteristics.
Does not appear to be harmful to
individual.

A

Bisalbuminemia (Genetic Origin)

82
Q

Extremely low or absent albumin

A

Analbuminemia (genetic origin)

83
Q

How is albumin measured?

A

-Chemical
-Electrophoresis
-Dye binding

84
Q

~90% of α1 fraction
- APR

A

α1-antitrypsin

85
Q

How is α1-antitrypsin measured?

A

Immunoassay**

86
Q

Normal range for α1-antitrypsin?

A

0.2-0.4 g/dl

87
Q

-neutralizes trypsin & trypsin-like enzymes
-protease inhibitor

A

α1-antitrypsin

“Neutrophil elastase” —> kills exogenous bacteria

88
Q

What happens with α1-antitrypsin deficiency?

A

severe, degenerative, EMPHYSEMATOUS PULMONARY DISEASE (damage to the alveoli that can cause respiratory acidosis) and juvenile hepatic cirrhosis ***

89
Q

How is α1-antitrypsin deficiency acquired?***

A

Chronic Liver Disease***
~ Severe protein deficiency
conditions

90
Q

What can cause increased α1-antitrypsin?

A

Acute Phase Reaction, pregnancy, oral contraceptive use

91
Q

Helps maintain the mucus layer in the first line of defense.

A

α1 ACID GLYCOPROTEIN (OROSOMUCOID) APR

92
Q

low pI(2.7) even in the acidic solution, negative charged

A

α1acid glycoprotein (Orosomucoid)

93
Q

maintaining mucus membrane integrity
cell membrane formation & fibers in association with collagen

A

α1acid glycoprotein (Orosomucoid)

94
Q

What increases α1acid glycoprotein ?

A

Acute Phase Reaction, pregnancy, cancer, pneumonia, RA,
conditions related to the cell proliferation*

95
Q

What causes decreased α1acid glycoprotein?

A

Inborn errors of metabolism

96
Q

How is α1acid glycoprotein measured?

A

Immunonephelometry, Immunofixation, Immunoassay(s)

97
Q

Why is α1acid glycoprotein not measured by SPE?

A

AAG: has high carb content, “NOT well stained in SPE”

98
Q

What is detected in the fetus and cancer?***

A

α1-FETOPROTEIN (AFP)***

99
Q

How is α1-FETOPROTEIN (AFP) measured?

A

**Immunoassay: RIA and EIA

100
Q

When does α1-FETOPROTEIN (AFP) decrease?

A

8-12 months after birth

101
Q

If α1-FETOPROTEIN (AFP) is detected in adults it means….

A

cancer (hepatic or gonad cancer)

-function not fully established in adult

102
Q

α1-FETOPROTEIN (AFP) is detectable maternal blood during pregnancy up to _____ months

A

7-8

103
Q

Used as screening test for several fetal conditions between 12 & 20 weeks
gestational age

A

α1-FETOPROTEIN (AFP)

104
Q

What conditions are associated with elevated AFP?

A

Anencephaly, Spina bifida, neural tube defects, atresia, fetal distress, ataxia-telangiectasia (Louis-Barr Syndrome)
Also increased in Twins

105
Q

What is associated with a low level of AFP?

A

3-4x increased risk for Down’s syndrome

106
Q

What is used as a tumor marker in adults?***

A

α1-FETOPROTEIN (AFP)

80% is hepatocellular carcinoma

107
Q

Any protein can be measured by ___________ if you have the antibody.

A

immunoassay

108
Q

High Density Lipoprotein (HDL) is part of the _________ fraction.

A

α1-Globulin

109
Q

Largest major nonimmunologic
circulating protein (mw 725 - 820 kD)

A

α2 MACROGLOBULIN

110
Q

What are the functions of α2 MACROGLOBULIN?

A

✴ Neutralize enzymes, [thrombin, trypsin, pepsin, plasmin] (blocks substrate binding site)
✴ Carrier for zinc
✴ Role in the innate or nonspecific immune response

111
Q

α2 MACROGLOBULIN is ___________ in nephrotic syndrome.

A

increased***

112
Q

How is α2 MACROGLOBULIN measured?

A

✴ Nephelometry, Immunoassay

113
Q

Coagulation factor II; converted to thrombin in process of coagulation; thrombin acts on fibrinogen to convert it to fibrin

A

PROTHROMBIN

114
Q

PROTHROMBIN is decreased in…

A

liver disease, Vitamin
K deficiency

115
Q

How is prothrombin measured?

A

prothrombin time

116
Q

What factors are vitamin K dependent?

A

2,7,9,10

117
Q

What is the function of thyroid binding globulin?

A

transport protein for T3 & T4
-major carrier

118
Q

How is thyroid binding globulin measured?

A

*Immunoassay

-Indirect measurement is by T3 Uptake (currently decreasing in use), TBG binding capacity

119
Q

What is the active form of iron?

A

ferric (Fe3+)

Fe3+ in food and gets converted to ferrous (Fe2+) to be absorbed and then to ferric (Fe3+) state to be used?

120
Q

bind free Hb to prevent loss of Hb via urine, and to transport it to the liver

A

Haptoglobin (APR)

121
Q

What can cause Haptoglobin to increase?

A

burns, nephrotic
syndrome, rheumatic disease, stress, infection, acute infection, tissue necrosis

122
Q

What can cause Haptoglobin to decrease?

A

IV hemolysis

123
Q

How is Haptoglobin measured? (must be free?)

A

✴ immunonephelometry,
✴ haptoglobin electrophoresis
✴ immunoassay

124
Q

✴ Transport protein for copper
✴ Enzymatic activities:
✓ Cu oxidase, histaminase,
ferroxidase

A

Ceruloplasmin APR

125
Q

What can cause Ceruloplasmin to increase?

A

inflammatory processes,
pregnancy, malignancies, oral estrogen therapy.

126
Q

What can cause Ceruloplasmin to be decreased?

A

Wilson’s Disease (increased copper in tissues and urine but decreased in blood),
Menkes’ kinky-hair syndrome

127
Q

How is Ceruloplasmin measured?

A

✴ Immunonephelometry
✴ immunoassay

128
Q

enzyme that catalyzes the oxidization of iron II to iron III

A

Ferroxidase is present in ceruloplamin

129
Q

Protein hormone produced in tubules of kidney (border of cortex and medulla)
✴ Stimulates erythropoiesis
-Acts on erythroid cell precursors in bone
marrow

A

Erythropoietin

130
Q

An potent cytoprotective (anti-apoptotic)
hormone

A

Erythropoietin

131
Q

■ Precursor to angiotensin I
■ After conversion to angiotensin II,
regulates, or increases blood
pressure via vasoconstriction

A

Angiotensinogen

132
Q

VLDL: What is the major fat carried?

A

TG (endogenous)

133
Q

Pre-beta?

A

α2 Lipoproteins (VLDL)?

134
Q

What is part of the α2-Globulin Fraction?

A

-α2 MACROGLOBULIN
-PROTHROMBIN
-THYROID BINDING GLOBULIN
-Haptoglobin
-Ceruloplasmin
-Erythropoietin
-Angiotensinogen

135
Q

What is part of the β-Globulin Fraction?

A

-β1 LIPOPROTEINS (LDL)
-FIBRINOGEN
-Plasminogen
-COMPLEMENT
-Transferrin (Dierophilin)
-C-1 ESTERASE INHIBITOR
-HEMOPEXIN
-β2 MICROGLOBULIN

136
Q

-Coagulation Factor I
■ Converted to fibrin by thrombin
■ Acute phase reactant

A

FIBRINOGEN

137
Q

What can cause increased fibrinogen?

A

inflammatory
conditions, pregnancy, oral
contraceptives

138
Q

How is fibrinogen measured?

A

coagulation

139
Q

Forms a distinct band between the β and γ areas when plasma is electrophoresed

A

Fibrinogen

140
Q

-Fibrinolytic protein activated to enzymatic status (plasmin) in coagulation process
-Plasmin slowly lyses fibrin clots

A

Plasminogen

141
Q

■ Fractions except C1 & C2
■ Enhances nonspecific cellular immune
response, such as phagocytosis,
anaphylaxis, lysis

A

COMPLEMENT

142
Q

What can cause decreased compliment?

A

malnutrition, DIC, SLE, RA,
recurrent infection

143
Q

How is compliment measured?

A

✴ immunonephelometry
✴ immunoassay
✴ CH50
✴ Total Hemolytic Complement

144
Q

Inhibits C-1 esterase (activated C-1)

A

C-1 ESTERASE INHIBITOR

145
Q

Deficiency in C-1 esterase inhibitor results in…

A

angioneurotic edema

146
Q

-Function is to bind free heme (decreases oxidative damage)
-Increased in acute phase
reaction

A

Hemopexin

147
Q

What decreases Hemopexin?

A

IV hemolysis &
hemolytic anemia

148
Q

How is Hemopexin measured?

A

✴ Nephelometry
✴ Immunoassay

149
Q

■ Light chain of HLA antigen
■ Found on surface of most nucleated cells, esp. high on lymphocytes

A

β2 MICROGLOBULIN

150
Q

MW ~11 kD
■ Filtered by renal glomerulus, but 99% is
reabsorbed and catabolized in the proximal tubules

A

β2 MICROGLOBULIN

151
Q

β2 MICROGLOBULIN is increased with

A

✴ tubular damage (poor clearance by kidney)
✴ Overproduction of certain cell types, such as occurs in certain inflammatory
diseases (RA, SLE)
-In HIV patients

152
Q

a high β2 level in the
absence of renal failure indicates…

A

a large lymphocyte turnover, suggesting the viral
killing of lymphocytes as seen with HIV patients

153
Q

How is β2 MICROGLOBULIN measured?

A

immunoassay

154
Q

What is part of the γ-Globulin Fraction?

A

*C1 & C2
-immunoglobulins (igG, igM, IgA, IgD, and IgE)

155
Q

■ Predominant immunoglobulin of the
secondary or anamnestic response
■ Crosses the placental barrier
■ Associated with long-term immunity
■ Highest concentration in blood of all immunoglobulins*
■ Usually reacts best at 37 degrees

A

IgG

156
Q

■ First immunoglobulin to be produced in immune response
■ Dominant immunoglobulin in primary
immune response
■ Pentamer structure
■ Largest immunoglobulin
■ Isohemagglutinin (Blood group antibodies)

A

IgM

157
Q

-Found both in blood and
on mucous membranes in
secretions
■ Exists as monomer and
dimer, possibly trimer
■ Associated with protection
of respiratory and
gastrointestinal disease

A

IgA

158
Q

■ Function in serum unknown
■ Found on surface of mature
B lymphocytes as part of
antigen recognition system
of B-cells
■ Very low concentration in
serum

A

IgD

159
Q

■ Associated with Type I
hypersentivities
■ Bound to mast cells in
tissues and basophils in
blood
■ Low concentration in serum

A

IgE

160
Q

Highest concentration in blood of all immunoglobulins

A

IgG