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
Q

What fraction do immunoglobulins migrate to and what are their function?

A

Gamma Globulins
Synsthesized by plasma cells associated with immune system

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26
Q

What protein, if detected on protein electrophoresis, could tell us that plasma was submitted instead of serum?

A

Fibrinogen - present as a distinct band between beta and gamma regions on plasma electrophoresis - WILL NOT be present in serum

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27
Q

What is considered the reference method for total protein measurement?

A

6.5-8.3 g/dL in serum

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28
Q

What are some causes of hypoproteinemia?

A

Excessive protein loss
Decreased Intake
Liver disease
Inherited immunodeficiency disorder
Accelerated catabolism of proteins

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29
Q

What are some causes of hyperproteinemia?

A

Dehydration
Presence of a paraprotein (multiple myeloma)
Polyclonal increases in immunoglobulins in many chronic disease

30
Q

What are some causes of hyperproteinemia?

A

Dehydration
Presence of a paraprotein (multiple myeloma)
Polyclonal increases in immunoglobulins in many chronic diseases

31
Q

What does the Biuret method measure?

A

Measures color proportional to number of peptide bonds present to calculate total protein

32
Q

What methodology do most laboratories use for albumin measurement?

A

Dye binding technique using bromocresol green/purple

33
Q

What might we perform protein electrophoresis on CSF for?

A

To detect oligoclonal bands- distinct bands seen in 90% of multiple sclerosis patients

34
Q

What element do proteins have that distinguish them from carbohydrates and lipids?

A

Nitrogen

35
Q

What does multiple myeloma do in the bone marrow and in the skeleton?

A

Osteolytic lesions

36
Q

What will someone with an alpha-antitrypsin deficiency electrophoresis pattern look like?

A

No Alpha-1 globulin peak

37
Q

What is the general pattern seen in inflammation?

A

Increase in alpha 1 and alpha 2; jagged looking peaks

38
Q

What will someone with cirrhosis electrophoresis pattern look like?

A

No break between beta and gamma - one long mountain-like peak.

39
Q

What will someone with nephrotic syndrome electrophoresis pattern look like?

A

Increased in alpha 2 and beta

40
Q

What will someone with a monoclonal increase electrophoresis pattern look like?

A

Large peak for gamma, taller than albumin - indicates overproduction of immunoglobulins such as seen in MM and WM

41
Q

What diseases are associated with an increase in CSF protein?

A

Bacterial/viral meningitis
Intracerebral hemorrhage
Multiple sclerosis
Traumatic tap

42
Q

What are the 4 main functions of the liver?

A

Excretion/secretion
Metabolism
Detoxification
Storage

43
Q

What enzyme is necessary for conjugating bilirubin?

A

UDPGT

44
Q

What (if anything) are conjugated, unconjugated, and delta bilirubin bound to?

A

Conjugated: bound to nothing
Unconjugated: bilirubin bound to albumin
Delta bilirubin: albumin bound to conjugated bilirubin

45
Q

The liver has a great reserve capacity. What percent of the liver can be destroyed before you even notice symptoms?

A

80%

46
Q

What is one of the largest products passed through the liver daily?

A

Bile

47
Q

Hemoglobin is broken down into:

A

Heme, Globin, and Iron

48
Q

What is the process for breaking down heme waste products?

A
  • 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
Q

What are conjugated and unconjugated bilirubin also known as?

A

Conjugated: direct bilirubin
Unconjugated: indirect bilirubin

50
Q

Define glycolysis

A

the breakdown of glucose

51
Q

Define glycogenolysis

A

the breakdown of glycogen into glucose

52
Q

Define gluconeogenesis

A

the production of glucose from non-carbohydrate sources

53
Q

What methodology is used to measure bilirubin?

A

Diazo reaction

54
Q

What accelerator does Malloy-Evelyn bilirubin technique use?

A

Methanol

55
Q

What accelerator does Jendrassik-Groff bilirubin technique use?

A

Caffeine

56
Q

What potentially fatal condition are we trying to prevent in newborns where bilirubin deposits in tissue?

A

Jaundice of the newborn - Kernicterus

57
Q

What are the typical components in a hepatic function panel?

A

Total protein, Albumin, Total bilirubin, Direct bilirubin, AST, ALT, ALP

58
Q

What is Gilbert’s syndrome?

A

Unconjugated bilirubinemia - genetic defect affecting production of UDPGT - have higher total bilirubin than average person.

59
Q

What is Crigler-Najjar?

A

Unconjugated bilirubinemia - has type 1 (no production of UDPGT) and type 2 (severe deficiency of UDPGT)

60
Q

What is jaundice of the newborn?

A

Unconjugated bilirubinemia - increased bilirubin load and/or lack of UDPGT production in newborns, can cause kernicterus leading to death

61
Q

What is Dubin-Johnson syndrome?

A

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
Q

What is Rotor syndrome?

A

Conjugated bilirubinemia - no dark granules seen in liver, but same as DJ syndrome (buildup of bilirubin in liver)

63
Q

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?

A

First-pass phenomenon

64
Q

What are prehepatic causes of jaundice?

A

PRE-LIVER: Acute and chronic hemolytic anemias (increased RBC destruction)

65
Q

What are hepatic causes of jaundice?

A

Intrinsic liver disease or defect

66
Q

What are posthepatic causes of jaundice?

A

AFTER LIVER: Biliary obstructive disease

67
Q

What tests can help identify hepatobiliary conditions (such as blockages)?

A

Enzyme tests; GGT will be elevated

68
Q

What are the five forms of infectious viral Hepatitis? how are they spread?

A

Hep A –> fecal-oral
Hep B –> percutaneous/permucosal
Hep C –> percutaneous/permucosal
Hep D –> percutaneous/permucosal
Hep E –> fecal-oral

69
Q

What is the 1st HBV marker we can detect?

A

Hep B surface antigen

70
Q

What HBV marker would ONLY we expect to see if the person is vaccinated, but not exposed to the real virus?

A

Hep B surface antibody

71
Q

prognosis of e antigen/antibody

A

better prognosis if e antibody is present
worse prognosis if only e antigen is present