66: Serum Proteins & Disorders Flashcards
The non-cellular liquid layer of the blood obtained by sedimentation and centrifugation.
Blood Plasma
Blood plasma prepared in a laboratory without blood coagulating factors
Blood Serum
Serum proteins are separated by ……….
Serum Protein Electrophoresis (SPEP)
Serum Protein Electrophoresis (SPEP) is often used with …
Densitometry: Identifies the amount and type of proteins in a serum sample
Proteins in the a1 globin band of densitometry:
- a1 antitrypsin
- a fetoprotein
- Transcortin
- Retinol binding protein
Proteins in the a2 globin band of densitometry:
- a2 macroglobulin
- Ceruloplasmin
- Haptoglobin
Proteins in the B globin band of densitometry:
- Transferrin
- Hemopexin
- LDL
Proteins in the gamma (y) globin band of densitometry:
Immunoglobulins IgG, IgM, IgA, IgD, IgE
Functions of Albumin:
- Maintenance of osmotic pressure
- Transport of Calcium ions, free fatty acids, bilirubin, hormones and drugs
Functions of a and B globulins:
Enzymes
Transport
Inhibitor Proteins
Functions of y-globulins:
Immune Response
………… is extensively synthesized in the liver (14 g/day) and is released into the blood.
Albumin
Albumin is small and the ……… abundant serum protein
Most
Normal serum range of Albumin:
3.5 - 5 g/dL
Individuals with congenital analbuminemia appear normal and do not show ………. Other serum proteins regulate the osmotic pressure in these individuals.
Edema
Hypoalbuminemia caused by decreased synthesis of albumin can lead to which diseases?
Kwashiorkor: Dietary deficiency of proteins and often infections
Liver Cirrhosis: Severe liver damage impairs synthesis of serum proteins
Hypoalbuminemia caused by increased loss of albumin can lead to which diseases?
Kidney disease: Loss into urine due to damage of glomerulus basement membrane
Severe burns: Damage of blood vessels leads to huge loss of serum
……….. inhibits neutrophil elastase in lung alveoli.
a1– Antitrypsin (a1-AT)
a1– Antitrypsin (a1-AT) is synthesized in ………….
Hepatocytes
Hereditary deficiency of the release of a1-ATinto the blood can lead to ………….. and …….. disease.
Pulmonary and liver disease
The reduced amount of a1-AT in the blood can lead to excessive degradation of ………. in the lung and can lead to emphysema
Elastin
Smoking activates neutrophils which release …………… and at the same time ROS modify the structure of a1-AT and reduce the binding to ……………..
Neutrophil elastase
Elevated neutrophil elastase also destroys ……… in the alveoli.
Elastin
………. is an a1 globulin that is abundant in fetal plasma which has very low levels of albumin
a fetoprotein (AFP)
AFP level is low in healthy adults but it increases in ……….
Cancer
Used as a serum marker for liver cancer
Maternal serum AFP is used as marker for possible ……….
Fetal abnormalities
…………. maternal serum AFP levels can be an indicator of neural tube defects
High
………… maternal serum AFP levels can be an indicator of Down syndrome.
Low
Transcortin transports 75% of ………… in blood.
Cortisol
Retinol-binding protein transports ………… in blood from the liver to the peripheral tissues
Retinol
……… is one of the largest serum proteins that inhibits an enormous variety of serum proteases like plasmin (fibrinolysis) and thrombin (coagulation).
a2 macroglobulin (A2M)
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.
Nephrotic Syndrome
………….. is a blue a2 globulin with mainly 2 functions:
- Copper transport in blood
- Ferroxidase activity
Ceruloplasmin
Ceruloplasmin is formed in the ……..binding of copper to apoceruloplasmin.
Liver
The released ceruloplasmin transports 95% of copper in blood.
Patients with …………. have very low blood levels of ceruloplasmin as hepatic copper binding is deficient
Wilson Disease
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.
Apoceruloplasmin
Damage due to …………. occurs in: the liver, brain, eyes and kidney.
Copper accumulation
Macrophages degrade heme and release ………..which could lead to radical formation (Fenton reaction)
Ferrous iron
Prevented by ceruloplasmin which forms ferric iron that can be bound to transferrin and transported in blood.
……….an a2-globulin that binds to free hemoglobin dimers in blood and prevents loss of Hb in urine.
Haptoglobin (Hp)
Haptoglobin-hemoglobin complex is taken up by ………….
Acute hemolysis leads to a low serum level of free haptoglobin.
Macrophages
Serum Protein Electrophoresis is used to monitor the progress in patients with …………….
Hemolytic anemia
…………is a b-globulin which transports ferric iron in blood between: intestine, liver, bone marrow and spleen.
Transferrin
Transferrin can bind two …….. atoms for transport.
Fe3+ (ferric ion)
Low transferrin saturation is found in patients with ………… as fewer sites of transferrin are filled
Iron deficiency
……….. transferrin saturation is found in patients with iron overload (…….. serum iron leads to increased binding)
High
……. is a b-globulin that binds to free heme in the blood and prevents the loss of heme-iron.
Hemopexin
Heme-hemopexin is taken up into ………… and the iron is bound mainly as ferric iron to the storage protein …………
Hepatocytes
Ferritin
Hemopexin prevents:
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.
The…….. globulin fraction is synthesized by plasma cells (activated B-lymphocytes).
y-globulin fraction
…….is found in blood and lymph and it is the first antibody to be produced in response to an antigen (infection).
IgM
……… (smallest and most common immunoglobulin) is found in all body fluids. It is produced by repeated exposure to the same antigen
IgG
IgG can cross the placenta giving passive immunity to the fetus and newborn.
……….is found in the lung, skin, mucous membranes and secreted in allergic reactions.
IgE
…….. is found in body secretions and protects body surfaces. It is found in human milk.
IgA
………..role in serum is uncertain
IgD
Acute phase reaction of the liver leads to
Changes in the synthesis of serum proteins
The …………. are serum proteins which are subdivided into positive and negative reactants depending on the amounts synthesized.
The acute phase reactants
Positive acute-phase reactants are synthesized in ………. amount as they reduce inflammation and deprive microbes of iron.
Larger amount
Negative acute-phase reactants are synthesized in ………amount in order to preserve amino acids for the increased synthesis of positive acute phase reactants
Smaller amount
Examples of Positive Acute-phase reactants:
- a1-antitrypsin
- Ceruloplasmin
- Haptoglobin
- Hemopexin
Examples of Negative Acute-Phase reactants:
- Albumin
- Transcortin
- Retinol-binding protein
- Transferrin
………. is an acute phase reactant that is synthesized and released during inflammation.
C-reactive protein (CRP)
Abnormal levels of albumin are related to:
Increased: Severe Dehydration
Reduced: Liver damage, Nephrotic syndrome, severe protein malnutrition and Acute phase response
Abnormal levels of a1-globulin are related to:
Increased: Acute and chronic inflammatory diseases, liver cancer (AFP).
Reduced: a1-AT deficiency, Nephrotic syndrome.
Abnormal levels of a2-globulin are related to:
Increased: Acute and chronic inflammatory diseases, Nephrotic syndrome (macroglobulin).
Reduced: Wilson disease (ceruloplasmin).
Abnormal levels of B-globulin are related to:
Increased: Hypercholesterolemia (LDL), Prolonged inflammation (hemopexin).
Reduced: Nephrotic syndrome.
Abnormal levels of y-globulin are related to:
Increased: acute and chronic inflammatory diseases, acute infections, liver cirrhosis, multiple myeloma and lymphoma.
Reduced: Hypogammaglobulinemia.
