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Liz's Clinical Chemistry Module 1 > Amino Acids, Proteins, and Analytical Methods > Flashcards

Amino Acids, Proteins, and Analytical Methods Flashcards

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1
Q

Amino acids may have a positive, negative, or neutral charges, depending on the ____

A

pH

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2
Q

Means that amino acids contain both acidic and basic groups

A

Amphoteric (zwitterion)

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3
Q

pH > 7.4, when a base is added to the amino acid resulting molecule has a ____ charge

A

Negative

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4
Q

pH = 7.4, internal transfer of a hydrogen ion from teh -COOH group the -NH2 group results in a ____ charge

A

Neutral

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5
Q

pH < 7.4, when an acid is added

A

Positive

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6
Q

Defined as the pH at which net charge is zero; i.e., there are equal numbers of positive and negative charges

A

Isoelectric point

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7
Q

If pH > pI

A

Amino acid has a negative charge

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8
Q

If pH < pI

A

Amino acid has a positive charge

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9
Q

Proteins act in vivo as ____ ____ due to their acid-base properties

A

mild buffer

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10
Q

Essential amino acids

A

Leucine, isoleucine, valine, methionine, tryptophan, phenylalanine, threonine, lysine

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11
Q

Formed when the alpha amino group of one amino acid is covalently linked w/ the alpha-carboxyl group of a second amino acid

A

Peptide bond

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12
Q

The formation of glucose from non-CHO sources, such as amino acids, glycerol, or fatty acids

A

Gluconeogenesis

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13
Q

Starting and ending products of gluconeogenesis

A

Starting: amino acids/glycerol/ fatty acids

Ending: glucose

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14
Q

The process of amino group removal or transfer to make different amino acids

A

Transamination

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15
Q

Starting and ending products of transamination (2 of them)

A

Starting: glutamic acid/oxaloacetate

Ending: alanine/aspartate

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16
Q

The removal of the amino group, forming toxic ammonia

A

Deamination

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17
Q

Starting and ending products of deamination

A

Starting: ammonia

Ending: urea

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18
Q

Organ in which the metabolic/catabolic pathways occur in the body

A

Liver

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19
Q

Occurs when the plasma levels of the amino acid are normal, but the renal reabsorptive mechanism is defective

A

Renal aminoaciduria

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20
Q

____ plasma levels and ____ urine levels of amino acids in renal aminoaciduria

A

normal; increased

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21
Q

Defect in renal tubular reabsorption of cystine, ornithine, lysine, and arginine; most common error of amino acid transport

A

Cystinuria

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22
Q

Three primary renal aminoacidurias

A

Cystinuria, Hartnup disease, and Fanconi syndrome

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23
Q

Prone to renal calculi of cystine; cystine crystals on urine microscopic

A

Lab findings in cystinuria

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24
Q

Increased urinary excretion of neutral monocarboxylic amino acids

A

Hartnup disease

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25
Q

Patients have a nicotinamide (vit B) deficiency, so they can have pellagra (red, scaly rash), plus neurological muscular abnormalities

A

Clinical features of Hartnup disease

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26
Q

Renal dysfunction of the proximal convoluted tubule

A

Fanconi Syndrome

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27
Q

Polyuria, dehydration, hypokalemia, acidosis, osteomalacia, cannot reabsorb necessary nutrients, bowing of the legs, low weight/height, abdominal distention, enlargement in wrist and ankle joints all indicate ____

A

Clinical features of Fanconi syndrome

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28
Q

Plasma level of one or more amino acids exceeds the renal threshold

A

Overflow aminoaciduria

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29
Q

____ plasma levels and ____ urine levels of amino acids in overal aminoaciduria

A

increased; increased

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30
Q

Due to a deficiency or absence of phenylalanine hydroxylase enzyme

A

Phenylketonuria (PKU)

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31
Q

Spastic movements and seizures, sever mental retardation if untreated, eczema, feeding difficulties, vomiting, delayed mental/social development, hypopigmentation, Increased serum phenylalanine, Decreased serum tyrosine, increased urine phenylpyruvic acid

A

Clinical features of PKU

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32
Q

Due to fumarylacetoacetase deficiency

A

Type I tyrosinemia

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33
Q

Causes cirrhosis and renal damage leading to Fanconi syndrome, elevated serum and urine tyrosine levels, elevated methionine in serum

A

Clinical features of Type I tyrosinemia

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34
Q

Due to tyrosine aminotransferase deficiency

A

Type II tyrosinemia

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35
Q

Skin lesions, ocular abnormalities (corneal dystrophy), elevated serum and urine levels of tyrosine, tyrosine crystals in urine, serium methionine NOT increased

A

Clinical features of type II tyrosinemia

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36
Q

Due to 4-hydroxyphenylpyruvate dioxygenase deficiency

A

Type III tyrosinemia

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37
Q

Mild mental retardation, seizures, periodic loss of balance and coordination, increased serum tyrosine

A

Type III tyrosinemia

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38
Q

Caused by a defect in the enzyme homogentisic acid oxidase

A

Alkaptonuria

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39
Q

Homogentisic acid increases in serum and urine, urine turns brown/black when exposed to light and air or when alkali is added, ocular ochronosis

A

Clinical features of alkaptonuria

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40
Q

Most commonly caused by a defect of CBS (cystathionine beta synthase)

A

Homocystinuria

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41
Q

Both methionine and homocystine are increased in serum and urine, ocular (lens displacement) and skeletal abnormalities (knock knee), cardiovascular problems (thromboli due to sticky platelets), pigeon chest

A

Clinical features of homocystinuria

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42
Q

Due to a defect in the enzyme alpha-ketoacid decarboxylase; also known as branched chain aminoaciduria due to excretion of the branched chain amino acids valine, leucine, and isoleucine

A

Maple Syrup Urine Disease (MSUD)

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43
Q

Hypoglycemia, ketacidosis (in blood and urine), lethargy, poor appetite, vomiting, convulsions, detected by the 11th day of life, maple syrup odor, increased ketone bodies

A

Clinical features of MSUD

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44
Q

Amino acids can be measured using 3 screening methods

A

Thin layer chromatography (TLC), photometric screening test, and Guthrie test

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45
Q

3 quantitative tests for the presence of aminoacidurias

A

Ion exchange chromatography, gas liquid chromatography (GLC), and high performance liquid chromatography (HPLC)

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46
Q

Four protein structures

A

Primary

Secondary

Tertiary

Quaternary

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47
Q

Specific sequence of amino acids

A

Primary structure

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48
Q

Uniting of several protein units or a protein plus another structure

A

Quaternary structure

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49
Q

Folding of a chain into a compact 3D conformation w/ a specific shape; confers specific biological properties

A

Tertiary structure

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50
Q

Recurring spatial arrangement of amino acids in 3D space

A

Secondary structure

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51
Q

Protein denaturation disrupts the bonds that hold ____, ____, and ____ structures together

A

Secondary, tertiary, and quaternary

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52
Q

What is happening when proteins lose their biological function?

A

Denaturation

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53
Q

4 processes that can denature proteins

A
  1. Extreme temperatures
  2. pH change
  3. Detergents, metals, organic solvents
  4. Mechanical mixing
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54
Q

8 biological functions of proteins

A
  1. Transport
  2. Cellular receptors
  3. Catalysis
  4. Structure
  5. Nutrition
  6. Maintenance of oncotic pressure
  7. Host defense
  8. Hormonal
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55
Q

Definition of “acute phase reactant” (APR)

A

Proteins that increase or decrease in response to an acute phase (inflammation, infection, myocardial infarction, tumor, surgery, trauma, etc.)

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56
Q

List 3 negative APRs

A

Transthyretin (prealbumin), albumin, transferrin

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57
Q

What is the importance of the different solubility characteristics of albumin and globulins?

A

Albumin is water-soluble

Globulins are water-insoluble

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58
Q

3 examples of fibrous proteins

A
  1. Collagen
  2. Elasin
  3. Keratin
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59
Q

Definition of nitrogen balance

A

Equals an equilibrium b/w intake and output

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60
Q

6 examples of conjugated proteins

A
  1. Nucleoproteins
  2. Mucoproteins
  3. Glycoproteins
  4. Lipoproteins
  5. Metalloproteins
  6. Phosphoproteins
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61
Q

What is positive nitrogen balance?

A

When intake > output

62
Q

How does positive nitrogen balance impact patient health?

A

It’s associated w/ general good health especially important in growing children, pregnant women, and body builders

63
Q

What is negative nitrogen balance?

A

When output > intake

64
Q

How does negative nitrogen balance impact patient health?

A

It’s seen in metastic cancer, severe infections, trauma, surgery, burns, and starvation

65
Q

2 names for the plasma protein commonly analyzed for nitrogen balance assessment

A

Transthyretin or prealbumin

66
Q

Hyperproteinemia caused by RELATIVE changes

A
  1. inadequate water intake
  2. Excessive water loss (severe vomiting, diarrhea)
67
Q

Two GENERAL processes which may cause total protein abnormalities

A
  1. Relative (apparent) change due to changes in water volume
  2. True (absolute) change
68
Q

Hyperproteinemia caused by ABSOLUTE changes

A
  1. Increased production (malignancy, such as multiple myeloma)
69
Q

Hypoproteinemia caused by RELATIVE changes

A
  1. Increased plasma water volume (water intoxication, massive IV infusion, salt retention syndromes)
70
Q

Hypoproteinemia caused by ABSOLUTE changes

A
  1. Increased loss (end-stage renal disease, such as nephrotic syndrome; severe blood loss; trauma; severe burns)
  2. Low protein intake or starvation
  3. Decreased production (liver disease)
71
Q

Definition of hemodilution

A

Increase in body water, causing an apparent decrease in plasma protein concentration

72
Q

Define hemoconcentration

A

Decrease in body water, causing an apparent increase in plasma protein concentration

73
Q

Function of albumin

A
  1. Transport of water-insoluble compounds
  2. Maintenance of colloid osmotic pressure
74
Q

Function of transthyretin (prealbumin)

A
  1. Indicator of nutritional status along w/ retinol binding protein (RBP)
  2. Transports thyroid hormones and retinol
75
Q

Albumin reference range (make sure to know units)

A

3.5–5.0 g/dL

76
Q

Hypoalbuminemia

  • 3 general causes
A
  1. Decreased production
  2. Decreased intake
  3. Increased loss
77
Q

4 examples of globular proteins

A
  1. Albumin
  2. Globulins
  3. Histones
  4. Protamine
78
Q

Causes for decreased production in hypoalbuminemia

A
  1. Liver disease
  2. Hereditary analbuminemia (rare)
79
Q

Causes for decreased intake in hypoalbuminemia

A
  1. GI disease
  2. Starvation
80
Q

Causes for increased loss in hypoalbumenia

A
  1. Renal disease
  2. Severe burns
81
Q

Names of 3 plasma proteins in alpha1-globulins

A
  1. alpha1 antitrypsin
  2. alpha1 acid glycoprotein
  3. alpha1 fetoprotein
82
Q

Functions of alpha1 antitrypsin

A

To inactivate protease enzymes (elastase, collagenase)

83
Q

Low levels of alpha1 antitrypsin is associated w/ what disease? High levels?

A

Low: juvenile-onset cirrhosis and emphysema due to M-protein deficiency

High: stress states

84
Q

Functions of alpha1 acid glycoprotein

A

To inactivate progesterone

85
Q

Alpha1 acid glycoprotein

  • Increased in what?
  • Decreased in what?
A

Increased in stress, rheumatoid arthritis, SLE (lupus), and Crohn’s disease

Decreased in malnutrition, severe liver damage, protien loss (GI and renal)

86
Q

Source of elevated alpha1 fetoprotein levels?

A

Synthesized in fetal liver and peaks in second trimester of pregnancy

87
Q

Alpha1 fetoprotein

  • Prenatal marker for neural tube defects increased in?
  • Prenatal marker decreased in?
A
  • Increased in spina bifida, anacephaly
  • Decreased in Down Syndrome
88
Q

List the 3 alpha2 globulins

A
  1. Haptoglobin
  2. Alpha2 macroglobulin
  3. Ceruloplasmin
89
Q

Function of haptoglobin

A

Binds to free hemoglobin in plasma in a ratio of 1 haptoglobin to 2 hemoglobins and transports it to RES to be degraded

90
Q

When is haptoglobin decreased?

A

Decreased in hemolytic anemia b/c it’s busy binding hemoglobin up

91
Q

Function of alpha2 macroglobulin

A

Functions as a protease inhibitor (trypsin, chymotrypsin, thrombin, etc.)

92
Q

Alpha2 macroglobulin is increased in what?

A

Increased in nephrotic syndrome

93
Q

Function of ceruloplasmin

A

Functions as a copper transporting protein

94
Q

Ceruloplasmin is increased in what? (5)

A
  1. Infections
  2. Malignancy
  3. Hodgkin’s disease
  4. Acute leukemia
  5. Rheumatoid arthritis
95
Q

Name the four beta globulins

A
  1. Transferrin
  2. Beta2 microglobulin
  3. Hemopexin
  4. C-reactive protein
96
Q

Function of transferrin

A

Functions to transport iron (and copper)

97
Q

Why is transferrin measured?

A

To diagnose and monitor iron deficiency anemia

98
Q

Transferrin

  • Increased in what?
  • Decreased in what?
A

increased in iron-deficiency anemia and pregnancy

Decreased in acute phase or protein-losing conditions

99
Q

Function of beta2 microglobulin

A

??

100
Q

Why is beta2 microglobulin measured?

A

To assess renal tubular function as it is small enough to be filtered by kidney

101
Q

Beta2 microglobulin

  • Increased in what?
  • Decreased in what?
A

Increased in neoplasm, especially those associated w/ B-lymphocytes

Decreased??

102
Q

Function of hemopexin

A

Binds heme after hemoglobin breakdown

  • Acts like a backup to haptoglobin
103
Q

What is the C-reactive protein?

A

Very sensitive but non-specific marker for systemic inflammation

104
Q

C-reactive protein

  • Elevated in what?
A

Elevated in persons w/ cardiovascular disease; strong predictor of future coronary events

105
Q

What are the relative risk categories for heart disease based on CRP predictions?

A
  1. Low risk
  2. Average risk
  3. High risk
106
Q

What are the functions of gamma globulins?

A

To respond to antigenic stimuli to recognize, destroy, and eliminate Ags

107
Q

Function of IgG

A

Functions as a major anti-viral and antibacterial Ab

108
Q

Functions of IgA

A

Functions to provide external surface protection against microorganisms

109
Q

Functions of IgM

A

Functions as the first immunoglobulin produced during an immune response

110
Q

Functions of IgD

A

May play a role in the activation of B-lymphocytes

111
Q

Functions of IgE

A

Associated w/ allergic (atopic) reactions (asthma, anaphylaxis, etc.)

112
Q

Clinical symptoms for multiple myeloma

A

Bone pain, punched out lesions on bone X-ray

113
Q

Lab findings associated w/ multiple myeloma

A
  • Plasma proteins increased (10-12)
  • Paraproteins present
  • Hyperviscosity of plasma
  • Bence-Jones protein in urine
  • 50% have increased IgG; 25% have increased IgA
114
Q

Specific source of paraprotein in multiple myeloma

A

malignant neoplasm involving a single clone of plasma cells

115
Q

Clinical symptoms for Waldenstom’s macroglobulinemia

A

??

116
Q

Lab findings associated w/ Waldenstom’s macroglobulinemia

A
  • increase in IgM from mature B-lymphocytes
  • Plasma hyperviscosity
  • 80% have Bence-Jones protein in urine
  • Rouleaux and plasmcytoid lymphs
117
Q

Specific source of paraprotein in Waldenstom’s macroglobulinemia

A

Arise from lymphoid tumors (lymphomas or chronic lymphocytic leukemia)

118
Q

Monoclonal gammopathy

A

Increase in one type of immunoglobulin only

119
Q

Polyclonal gammopathy

A
  • Due to infection
  • Increase in IgG in autoimmune response
  • Increase in IgA in skin, respiratory, or renal infections
  • Increase in IgM in viral infections or malaria
120
Q

Hypogammaglobulinemia

A
  • Lack of one or more immunoglobulins due to B-lymphocyte malfunction
  • Highly susceptible to bacterial infections
  • Treat w/ IV immunoglobulins
121
Q

Agammaglobulinemia

A
  • Due to genetic defect
  • Decrease of IgG
  • Recurrent infections
  • Treat w/ immunoglobulins, antibiotics, BMT
122
Q

Plasma protein reference range (make sure to know the units)

A

6.4–8.3 g/dL

123
Q

Ceruplasmin

  • Decreased in what (3)?
A
  1. Wilson’s disease
  2. Malnutrition
  3. Chronic hepatitis
124
Q

Reference range for total protein (make sure to know units)

A

6.8–8.3 g/dL

125
Q

Reference range for albumin (make sure to know units)

A

3.4–5.0 g/dL

126
Q

This analytic method is based on quantitation of the nitrogen content of proteins

  • Nitrogen content x 6.25 to obtain total protein value
A

Kjeldahl Method

  • Dr. Jim’s BIG WHOOSH!!
127
Q

In the presence of cupric ions, this test yields a violet color; intensity of color produced is proportional to the number of peptide bonds present to bind w/ cupric ions in alkaline solution

A

Biuret Method

128
Q

Based on the refractive index of light; ratio of light refraction in two differing media is related to protein concentration

A

Refractometry

129
Q

Dye-binding method using bromocresol green or bromocresol purple

A

Albumin Method

130
Q

Quantitative passie diffusion, Ag in serum diffuses from well until Ab excess causes immune precipitation, observed as ring around well

A

Radial immunodiffusion (RID)

131
Q

All antigenic sites are covered w/ Ab and lattic formation is inhibited; more Ab than Ag

A

Prozone, Ab excess

132
Q

Optimal proportion of Ab and Ag; 2-3 molecules are present for each Ag moleucle; maximum lattice formation and maximum precipitate; insoluble complexes

A

Equivalence zone

133
Q

Ag excess; all Ab sites are saturated by Ag; more Ag than Ab

A

Postzone

134
Q

The migration of charged solutes or particles (proteins) in a liquid medium under the influence of an applied external electrical field

A

Electrophoresis

135
Q

3 support media for electrophoresis

A
  1. Agarose gel
  2. Cellulose acetate
  3. Polyacrylamide gel
136
Q

Acidic pH = protein is a ____

A

Cation

137
Q

The cation migrates to the ____, which is a ____ pole

A

Cathod; negative

138
Q

Alkaline pH = protein is an ____, and migrates to the anode, which is a ____ pole

A

Anion; positive

139
Q

Migration pattern from anode to cathode for serum protein electrophoresis

A

Albumin, then alpha1 globulins, then alpha2 globulins, then beta globulins, then gamma globulins

140
Q

The following electrophoresis data describes ____

↓ albumin and beta regions

↑ alpha1 and alpha2 regions

Overall ↓ total protein

A

Acute inflammation

141
Q

The following electrophoresis data describes ____

↓ albumin

↑ alpha1, alpha2, beta, gamma globulins

Overall ↑ total protein

A

Chronic inflammation

142
Q

The following electrophresis data describes ____

↑ alpha2 and beta globulins

only 4 peaks causing a beta-gamma bridge followed by an M-spike

A

Cirrhosis

143
Q

The following electrophoresis data describes ____

↓ albumin, alpha1, beta, gamma globulins

↑ alpha2 globulins

Overall ↓ total protein

A

Nephrotic syndrome

144
Q

The following electrophoresis data describes ____

↓ gamma globulins

A

Hypogammaglobulinemia

145
Q

The following electrophoresis data describes ____

↑ gamma globulins causing an M-spike

Overall ↑ total protein

A

Hypergammagloulinemia (monoclonal gammopathy)

146
Q

The following electrophoresis data describes ____

↑ gamma region

A

Polyclonal gammopathy (diffuse increase in gamma region)

147
Q

The following electrophoresis data describes ____

↓ alpha1 region

A

Alpha1 antitrypsin deficiency

148
Q

2 causes of increased CSF total protein

A
  1. Increased endogenous production (i.e, MS)
  2. Damage to BBB causing leakage of blood into CNS (infections, stroke, skull fracture, etc)
149
Q

About 90% of patients w/ MS have a(n) ____ CSF IgG index ( > 0.70)

  • What is the calculation to find the CSF IgG index?
A

Increased

(IgGCSF x albuminserum) / (IgGserum x albuminCSF)

150
Q

The synthesis of complex molecules in living organisms from simpler ones together w/ the storage of energy

A

Anabolism

151
Q

The breakdown of complex molecules in living organisms to form simpler ones, together w/ the release of energy

A

Catabolism

Liz's Clinical Chemistry Module 1 (5 decks)

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