66: Serum Proteins & Disorders Flashcards

1
Q

The non-cellular liquid layer of the blood obtained by sedimentation and centrifugation.

A

Blood Plasma

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2
Q

Blood plasma prepared in a laboratory without blood coagulating factors

A

Blood Serum

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3
Q

Serum proteins are separated by ……….

A

Serum Protein Electrophoresis (SPEP)

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4
Q

Serum Protein Electrophoresis (SPEP) is often used with …

A

Densitometry: Identifies the amount and type of proteins in a serum sample

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5
Q

Proteins in the a1 globin band of densitometry:

A
  • a1 antitrypsin
  • a fetoprotein
  • Transcortin
  • Retinol binding protein
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6
Q

Proteins in the a2 globin band of densitometry:

A
  • a2 macroglobulin
  • Ceruloplasmin
  • Haptoglobin
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7
Q

Proteins in the B globin band of densitometry:

A
  • Transferrin
  • Hemopexin
  • LDL
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8
Q

Proteins in the gamma (y) globin band of densitometry:

A

Immunoglobulins IgG, IgM, IgA, IgD, IgE

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9
Q

Functions of Albumin:

A
  • Maintenance of osmotic pressure

- Transport of Calcium ions, free fatty acids, bilirubin, hormones and drugs

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10
Q

Functions of a and B globulins:

A

Enzymes
Transport
Inhibitor Proteins

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11
Q

Functions of y-globulins:

A

Immune Response

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12
Q

………… is extensively synthesized in the liver (14 g/day) and is released into the blood.

A

Albumin

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13
Q

Albumin is small and the ……… abundant serum protein

A

Most

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14
Q

Normal serum range of Albumin:

A

3.5 - 5 g/dL

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15
Q

Individuals with congenital analbuminemia appear normal and do not show ………. Other serum proteins regulate the osmotic pressure in these individuals.

A

Edema

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16
Q

Hypoalbuminemia caused by decreased synthesis of albumin can lead to which diseases?

A

Kwashiorkor: Dietary deficiency of proteins and often infections

Liver Cirrhosis: Severe liver damage impairs synthesis of serum proteins

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17
Q

Hypoalbuminemia caused by increased loss of albumin can lead to which diseases?

A

Kidney disease: Loss into urine due to damage of glomerulus basement membrane

Severe burns: Damage of blood vessels leads to huge loss of serum

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18
Q

……….. inhibits neutrophil elastase in lung alveoli.

A

a1– Antitrypsin (a1-AT)

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19
Q

a1– Antitrypsin (a1-AT) is synthesized in ………….

A

Hepatocytes

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20
Q

Hereditary deficiency of the release of a1-ATinto the blood can lead to ………….. and …….. disease.

A

Pulmonary and liver disease

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21
Q

The reduced amount of a1-AT in the blood can lead to excessive degradation of ………. in the lung and can lead to emphysema

A

Elastin

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22
Q

Smoking activates neutrophils which release …………… and at the same time ROS modify the structure of a1-AT and reduce the binding to ……………..

A

Neutrophil elastase

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23
Q

Elevated neutrophil elastase also destroys ……… in the alveoli.

A

Elastin

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24
Q

………. is an a1 globulin that is abundant in fetal plasma which has very low levels of albumin

A

a fetoprotein (AFP)

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25
Q

AFP level is low in healthy adults but it increases in ……….

A

Cancer

Used as a serum marker for liver cancer

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26
Q

Maternal serum AFP is used as marker for possible ……….

A

Fetal abnormalities

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27
Q

…………. maternal serum AFP levels can be an indicator of neural tube defects

A

High

28
Q

………… maternal serum AFP levels can be an indicator of Down syndrome.

A

Low

29
Q

Transcortin transports 75% of ………… in blood.

A

Cortisol

30
Q

Retinol-binding protein transports ………… in blood from the liver to the peripheral tissues

A

Retinol

31
Q

……… is one of the largest serum proteins that inhibits an enormous variety of serum proteases like plasmin (fibrinolysis) and thrombin (coagulation).

A

a2 macroglobulin (A2M)

32
Q

A greater than 10 fold increase (abnormal) in a2 macroglobulin blood is often found in patients with …………. due to damage of the basement membrane of the glomerulus.

A

Nephrotic Syndrome

33
Q

………….. is a blue a2 globulin with mainly 2 functions:

  1. Copper transport in blood
  2. Ferroxidase activity
A

Ceruloplasmin

34
Q

Ceruloplasmin is formed in the ……..binding of copper to apoceruloplasmin.

A

Liver

The released ceruloplasmin transports 95% of copper in blood.

35
Q

Patients with …………. have very low blood levels of ceruloplasmin as hepatic copper binding is deficient

A

Wilson Disease

36
Q

Patients with Wilson Disease have a deficiency of a copper-transporting ATPase (ATPase7-B) which is needed to link copper to ……….. and it is also needed for release of copper into bile.

A

Apoceruloplasmin

37
Q

Damage due to …………. occurs in: the liver, brain, eyes and kidney.

A

Copper accumulation

38
Q

Macrophages degrade heme and release ………..which could lead to radical formation (Fenton reaction)

A

Ferrous iron

Prevented by ceruloplasmin which forms ferric iron that can be bound to transferrin and transported in blood.

39
Q

……….an a2-globulin that binds to free hemoglobin dimers in blood and prevents loss of Hb in urine.

A

Haptoglobin (Hp)

40
Q

Haptoglobin-hemoglobin complex is taken up by ………….

Acute hemolysis leads to a low serum level of free haptoglobin.

A

Macrophages

41
Q

Serum Protein Electrophoresis is used to monitor the progress in patients with …………….

A

Hemolytic anemia

42
Q

…………is a b-globulin which transports ferric iron in blood between: intestine, liver, bone marrow and spleen.

A

Transferrin

43
Q

Transferrin can bind two …….. atoms for transport.

A

Fe3+ (ferric ion)

44
Q

Low transferrin saturation is found in patients with ………… as fewer sites of transferrin are filled

A

Iron deficiency

45
Q

……….. transferrin saturation is found in patients with iron overload (…….. serum iron leads to increased binding)

A

High

46
Q

……. is a b-globulin that binds to free heme in the blood and prevents the loss of heme-iron.

A

Hemopexin

47
Q

Heme-hemopexin is taken up into ………… and the iron is bound mainly as ferric iron to the storage protein …………

A

Hepatocytes

Ferritin

48
Q

Hemopexin prevents:

A

A. Heme-induced damage of plasma membranes by heme insertion and hydroxyl radical formation.
B. Heme usage by microbes: heme is an important source of iron for pathogenic microorganisms.

49
Q

The…….. globulin fraction is synthesized by plasma cells (activated B-lymphocytes).

A

y-globulin fraction

50
Q

…….is found in blood and lymph and it is the first antibody to be produced in response to an antigen (infection).

A

IgM

51
Q

……… (smallest and most common immunoglobulin) is found in all body fluids. It is produced by repeated exposure to the same antigen

A

IgG

IgG can cross the placenta giving passive immunity to the fetus and newborn.

52
Q

……….is found in the lung, skin, mucous membranes and secreted in allergic reactions.

A

IgE

53
Q

…….. is found in body secretions and protects body surfaces. It is found in human milk.

A

IgA

54
Q

………..role in serum is uncertain

A

IgD

55
Q

Acute phase reaction of the liver leads to

A

Changes in the synthesis of serum proteins

56
Q

The …………. are serum proteins which are subdivided into positive and negative reactants depending on the amounts synthesized.

A

The acute phase reactants

57
Q

Positive acute-phase reactants are synthesized in ………. amount as they reduce inflammation and deprive microbes of iron.

A

Larger amount

58
Q

Negative acute-phase reactants are synthesized in ………amount in order to preserve amino acids for the increased synthesis of positive acute phase reactants

A

Smaller amount

59
Q

Examples of Positive Acute-phase reactants:

A
  • a1-antitrypsin
  • Ceruloplasmin
  • Haptoglobin
  • Hemopexin
60
Q

Examples of Negative Acute-Phase reactants:

A
  • Albumin
  • Transcortin
  • Retinol-binding protein
  • Transferrin
61
Q

………. is an acute phase reactant that is synthesized and released during inflammation.

A

C-reactive protein (CRP)

62
Q

Abnormal levels of albumin are related to:

A

Increased: Severe Dehydration

Reduced: Liver damage, Nephrotic syndrome, severe protein malnutrition and Acute phase response

63
Q

Abnormal levels of a1-globulin are related to:

A

Increased: Acute and chronic inflammatory diseases, liver cancer (AFP).

Reduced: a1-AT deficiency, Nephrotic syndrome.

64
Q

Abnormal levels of a2-globulin are related to:

A

Increased: Acute and chronic inflammatory diseases, Nephrotic syndrome (macroglobulin).

Reduced: Wilson disease (ceruloplasmin).

65
Q

Abnormal levels of B-globulin are related to:

A

Increased: Hypercholesterolemia (LDL), Prolonged inflammation (hemopexin).

Reduced: Nephrotic syndrome.

66
Q

Abnormal levels of y-globulin are related to:

A

Increased: acute and chronic inflammatory diseases, acute infections, liver cirrhosis, multiple myeloma and lymphoma.

Reduced: Hypogammaglobulinemia.