Exam 6: Proteins/Liver Function/Nonprotein nitrogen Flashcards

1
Q

What is the primary structure of a protein?

A

Sequence of amino acids

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2
Q

What is the secondary structure of a protein?

A

Formation of a helix

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3
Q

What is the tertiary structure of a protein?

A

Folded 3D protein structure

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4
Q

What is the quaternary structure of a protein?

A

Two or more peptide chains integrated

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5
Q

What two enzymes digest dietary proteins?

A

Pepsin and trypsin

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6
Q

PKU: cause, urine characteristics

A

Buildup of phenylalanine in the blood causes urine to have a musty/mousy odor

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7
Q

What is the Guthrie test?

A

Used to detect PKU; if elevated levels of phenylalanine are found in the blood on the filter paper, bacteria will grow on agar plate

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8
Q

What can tyrosinemia lead to?

A

liver damage

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9
Q

Alkaptonuria

A

Accumulation of homogentisic acid turns urine black colored

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10
Q

Maple Syrup Urine Disease

A

Blocks normal metabolism of leucine, isoleucine, and valine that causes urine to smell like maple syrup

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11
Q

What is a proteins isoelectric point?

A

The pH at which an amino acid or protein has no net charge

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12
Q

Most proteins are synthesized in the ______.

A

liver

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13
Q

What are some functions of proteins?

A

Tissue nutrition
Maintain oncotic pressure
Act as buffers to maintain pH
Transportation of metabolic substances
Immunity (antibodies)
Hormones and receptors
Structure (connective tissue)
Enzymes
Coagulation and hemostasis

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14
Q

What fraction does prealbumin migrate to in electrophoresis and what is its function?

A

Just ahead of albumin - rarely distinguished from albumin band. Prealbumin transports the thyroid hormones T3 and T4 and Retinol (vitamin A)

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15
Q

What fraction does albumin migrate to in electrophoresis and what is its function?

A

It is the first large peak (band). This is the protein in highest concentration in the blood.
It binds and transports materials in the bloodstream and maintains oncotic pressure/fluid balance in tissues.

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16
Q

Analbuminemia vs bisalbuminemia

A

Do not produce any albumin vs produces two types of albumin

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17
Q

What fraction does alpha-1 antitrypsin migrate to and what is its function?

A

Alpha-1 Globulins
Functions to neutralize enzymes that cause damage to structural protein in the lungs

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18
Q

What fraction does alpha-1 fetoprotein migrate to and what is its function?

A

Alpha-1 Globulins
Synthesized by fetal yolk sac - Useful for screening for any conditions where fetus produces excess proteins or insufficient proteins and useful for tumor markers

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19
Q

What fraction does haptoglobin migrate to and what is its function?

A

Alpha-2 Globulins
Functions to bind free hemoglobin

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20
Q

What fraction does ceruloplasmin migrate to and what is its function?

A

Alpha-2 Globulins
Contains 90% of body’s copper and has enzymatic activities

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21
Q

What is Wilson’s disease and what protein is used to help diagnose it?

A

Inherited disorder where copper is deposited in the skin, liver, cornea, and brain presenting with low ceruloplasmin levels

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22
Q

What fraction does Alpha-2 Macroglobulin migrate to and what is its function?

A

Alpha-2 Globulins
Inhibits proteases such as trypsin, thrombin, kallikrein, and plasmin

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23
Q

What fraction does Transferrin migrate to and what is its function?

A

Beta-Globulins
Functions to transport iron

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24
Q

What fraction does C-Reactive Protein migrate to and what is its function?

A

Beta-Globulins
Elevated in situations of inflammation, used as a risk factor for cardiovascular disease

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25
What fraction do immunoglobulins migrate to and what are their function?
Gamma Globulins Synsthesized by plasma cells associated with immune system
26
What protein, if detected on protein electrophoresis, could tell us that plasma was submitted instead of serum?
Fibrinogen - present as a distinct band between beta and gamma regions on plasma electrophoresis - WILL NOT be present in serum
27
What is considered the reference method for total protein measurement?
6.5-8.3 g/dL in serum
28
What are some causes of hypoproteinemia?
Excessive protein loss Decreased Intake Liver disease Inherited immunodeficiency disorder Accelerated catabolism of proteins
29
What are some causes of hyperproteinemia?
Dehydration Presence of a paraprotein (multiple myeloma) Polyclonal increases in immunoglobulins in many chronic disease
30
What are some causes of hyperproteinemia?
Dehydration Presence of a paraprotein (multiple myeloma) Polyclonal increases in immunoglobulins in many chronic diseases
31
What does the Biuret method measure?
Measures color proportional to number of peptide bonds present to calculate total protein
32
What methodology do most laboratories use for albumin measurement?
Dye binding technique using bromocresol green/purple
33
What might we perform protein electrophoresis on CSF for?
To detect oligoclonal bands- distinct bands seen in 90% of multiple sclerosis patients
34
What element do proteins have that distinguish them from carbohydrates and lipids?
Nitrogen
35
What does multiple myeloma do in the bone marrow and in the skeleton?
Osteolytic lesions
36
What will someone with an alpha-antitrypsin deficiency electrophoresis pattern look like?
No Alpha-1 globulin peak
37
What is the general pattern seen in inflammation?
Increase in alpha 1 and alpha 2; jagged looking peaks
38
What will someone with cirrhosis electrophoresis pattern look like?
No break between beta and gamma - one long mountain-like peak.
39
What will someone with nephrotic syndrome electrophoresis pattern look like?
Increased in alpha 2 and beta
40
What will someone with a monoclonal increase electrophoresis pattern look like?
Large peak for gamma, taller than albumin - indicates overproduction of immunoglobulins such as seen in MM and WM
41
What diseases are associated with an increase in CSF protein?
Bacterial/viral meningitis Intracerebral hemorrhage Multiple sclerosis Traumatic tap
42
What are the 4 main functions of the liver?
Excretion/secretion Metabolism Detoxification Storage
43
What enzyme is necessary for conjugating bilirubin?
UDPGT
44
What (if anything) are conjugated, unconjugated, and delta bilirubin bound to?
Conjugated: bound to nothing Unconjugated: bilirubin bound to albumin Delta bilirubin: albumin bound to conjugated bilirubin
45
The liver has a great reserve capacity. What percent of the liver can be destroyed before you even notice symptoms?
80%
46
What is one of the largest products passed through the liver daily?
Bile
47
Hemoglobin is broken down into:
Heme, Globin, and Iron
48
What is the process for breaking down heme waste products?
- Destroyed RBCs are broken down into heme. - - Heme gets broken down into bilirubin - Albumin binds bilirubin and turns it into unconjugated bilirubin - UDPGT converts unconjugated bilirubin into conjugated bilirubin in the liver and it becomes more soluble - GI bacteria convert conjugated bilirubin into urobilinogen in the intestines - Urobilinogen excreted in feces
49
What are conjugated and unconjugated bilirubin also known as?
Conjugated: direct bilirubin Unconjugated: indirect bilirubin
50
Define glycolysis
the breakdown of glucose
51
Define glycogenolysis
the breakdown of glycogen into glucose
52
Define gluconeogenesis
the production of glucose from non-carbohydrate sources
53
What methodology is used to measure bilirubin?
Diazo reaction
54
What accelerator does Malloy-Evelyn bilirubin technique use?
Methanol
55
What accelerator does Jendrassik-Groff bilirubin technique use?
Caffeine
56
What potentially fatal condition are we trying to prevent in newborns where bilirubin deposits in tissue?
Jaundice of the newborn - Kernicterus
57
What are the typical components in a hepatic function panel?
Total protein, Albumin, Total bilirubin, Direct bilirubin, AST, ALT, ALP
58
What is Gilbert's syndrome?
Unconjugated bilirubinemia - genetic defect affecting production of UDPGT - have higher total bilirubin than average person.
59
What is Crigler-Najjar?
Unconjugated bilirubinemia - has type 1 (no production of UDPGT) and type 2 (severe deficiency of UDPGT)
60
What is jaundice of the newborn?
Unconjugated bilirubinemia - increased bilirubin load and/or lack of UDPGT production in newborns, can cause kernicterus leading to death
61
What is Dubin-Johnson syndrome?
Conjugated bilirubinemia - deficiency of transporter protein that moves bilirubin out of liver so bilirubin cannot be excreted from liver. buildup of delta bilirubin (albumin bound to conjugated bilirubin)
62
What is Rotor syndrome?
Conjugated bilirubinemia - no dark granules seen in liver, but same as DJ syndrome (buildup of bilirubin in liver)
63
What is the name of the phenomenon where virtually all ingested materials are taken to the liver for evaluation prior to widespread circulation in the body?
First-pass phenomenon
64
What are prehepatic causes of jaundice?
PRE-LIVER: Acute and chronic hemolytic anemias (increased RBC destruction)
65
What are hepatic causes of jaundice?
Intrinsic liver disease or defect
66
What are posthepatic causes of jaundice?
AFTER LIVER: Biliary obstructive disease
67
What tests can help identify hepatobiliary conditions (such as blockages)?
Enzyme tests; GGT will be elevated
68
What are the five forms of infectious viral Hepatitis? how are they spread?
Hep A --> fecal-oral Hep B --> percutaneous/permucosal Hep C --> percutaneous/permucosal Hep D --> percutaneous/permucosal Hep E --> fecal-oral
69
What is the 1st HBV marker we can detect?
Hep B surface antigen
70
What HBV marker would ONLY we expect to see if the person is vaccinated, but not exposed to the real virus?
Hep B surface antibody
71
prognosis of e antigen/antibody
better prognosis if e antibody is present worse prognosis if only e antigen is present