Genetic Polymorphism and α1-Antitrypsin
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Questions and Answers

Which of the following is true about the difference between serum and plasma?

  • Serum is obtained when blood is centrifuged in the presence of anticoagulant
  • Plasma is more commonly used in clinical laboratories
  • Serum contains fibrinogen while plasma does not
  • Plasma is obtained when blood is centrifuged in the presence of anticoagulant (correct)

    • where are y proteins are synthesized?

  • in fibrinogen
  • in liver enzymes
  • in formal blood cells
  • in plasma cells (correct)

    • Why are blood collection tubes color-coded?

  • To indicate the substances they contain
  • To indicate the type of anticoagulant used (correct)
  • To facilitate blood collection
  • To differentiate between serum and plasma

    • Which of the following methods is commonly used to analyze plasma proteins?

    <p>Radioimmunoassay</p> Signup and view all the answers

    Which plasma protein is responsible for binding and transporting thyroid hormones?

    <p>Transthyretin</p> Signup and view all the answers

    What is not one of the functions of albumin in plasma?

    <p>all are true</p> Signup and view all the answers

    Which of the following diseases is associated with a decreased albumin-globulin ratio in the plasma?

    <p>Liver diseases</p> Signup and view all the answers

    Which protein is responsible for binding free heme in the plasma?

    <p>Hemopexin</p> Signup and view all the answers

    What is the total iron-binding capacity (TIBC) of plasma?

    <p>300 μg of iron</p> Signup and view all the answers

    Which type of amyloidosis is typically due to a monoclonal plasma cell disorder?

    <p>Primary amyloidosis</p> Signup and view all the answers

    How many antigen-binding fragments (Fab) are produced when the hinge region of an immunoglobulin is digested by papain?

    <p>Two</p> Signup and view all the answers

    Which protein carries 90% of the copper in plasma?

    <p>Ceruloplasmin</p> Signup and view all the answers

    Which protein forms a tight noncovalent complex with extracorpuscular hemoglobin?

    <p>Haptoglobin</p> Signup and view all the answers

    Which protein is responsible for inhibiting serine proteases?

    <p>α1-Antitrypsin</p> Signup and view all the answers

    Which protein transports 10% of zinc in plasma?

    <p>α2-Macroglobulin</p> Signup and view all the answers

    Which of the following is true about the light chains in immunoglobulins?

    <p>There are two general types of light chains: kappa (κ) and lambda (λ).</p> Signup and view all the answers

    Which of the following statements about immunoglobulin molecules is correct?

    <p>A given immunoglobulin molecule always contains either two kappa or two lambda light chains.</p> Signup and view all the answers

    Which of the following is true about the heavy chains in immunoglobulins?

    <p>There are five types of heavy chains: alpha (α), beta (β), gamma (γ), delta (δ), and epsilon (ε).</p> Signup and view all the answers

    Which of the following is a characteristic of monoclonal gammopathies?

    <p>They are associated with a sharp peak on plasma protein electrophoresis called a paraprotein.</p> Signup and view all the answers

    Which gene is mutated in Menkes Disease?

    <p>ATP7A</p> Signup and view all the answers

    What is the characteristic feature of Menkes Disease?

    <p>Kinky or steely hair</p> Signup and view all the answers

    Which organ expresses very little of the copper binding P-type ATPase?

    <p>Liver</p> Signup and view all the answers

    Which of the following cells is NOT a main source of α2-macroglobulin in the body?

    <p>Neutrophils</p> Signup and view all the answers

    What is the reactive bond responsible for the biological actions of α2-macroglobulin?

    <p>Cyclic thiol ester bond</p> Signup and view all the answers

    Which of the following is NOT a function of α2-macroglobulin?

    <p>Promoting blood clotting</p> Signup and view all the answers

    Which of the following is true about haptoglobin (Hp)?

    <p>Hp is a plasma protein that binds to hemoglobin and prevents the loss of free hemoglobin into the kidney</p> Signup and view all the answers

    Which of the following is the molecular mass of the Hb-Hp complex?

    <p>155 kDa</p> Signup and view all the answers

    Why does the Hb-Hp complex prevent the loss of free hemoglobin into the kidney?

    <p>The Hb-Hp complex is too large to pass through the glomerulus</p> Signup and view all the answers

    Which of the following is a characteristic of hemolytic anemia?

    <p>Hemolytic anemia is caused by low levels of Hp.</p> Signup and view all the answers

    What is the role of haptoglobin (Hp) in the body?

    <p>Hp binds and removes free hemoglobin from the plasma.</p> Signup and view all the answers

    Which of the following is a possible consequence of low levels of Hp in the body?

    <p>Increased risk of kidney damage.</p> Signup and view all the answers

    Which protein is responsible for shuttling iron to sites where it is needed?

    <p>Transferrin</p> Signup and view all the answers

    Why is free iron extremely toxic in biological systems?

    <p>It forms reactive oxygen species (ROS)</p> Signup and view all the answers

    Why is iron always bound to proteins in biological systems?

    <p>To limit reactive oxygen species formation</p> Signup and view all the answers

    Which protein is responsible for binding and transporting iron in the plasma?

    <p>Transferrin</p> Signup and view all the answers

    What is the total iron-binding capacity (TIBC) of plasma?

    <p>300 μg/dL</p> Signup and view all the answers

    In which organ is transferrin synthesized?

    <p>Liver</p> Signup and view all the answers

    Which of the following conditions can impair glycosylation of transferrin?

    <p>Both congenital disorders of glycosylation and chronic alcoholism</p> Signup and view all the answers

    What is the role of transferrin in the body?

    <p>Transporting iron to sites where it is needed</p> Signup and view all the answers

    Which of the following conditions can impair glycosylation of transferrin?

    <p>Both congenital disorders of glycosylation and chronic alcoholism</p> Signup and view all the answers

    Which of the following is true about ferritin?

    <p>Ferritin is composed of 24 subunits</p> Signup and view all the answers

    What is the function of ferritin in the body?

    <p>Ferritin stores excess iron in various tissues</p> Signup and view all the answers

    What do plasma ferritin levels indicate?

    <p>Plasma ferritin levels indicate body iron stores</p> Signup and view all the answers

    Which plasma protein binds free heme?

    <p>Hemopexin</p> Signup and view all the answers

    Which plasma protein forms methalbumin by binding metheme?

    <p>Albumin</p> Signup and view all the answers

    Which plasma protein transfers metheme to hemopexin?

    <p>Albumin</p> Signup and view all the answers

    Which cells are responsible for the synthesis of circulating humoral antibodies?

    <p>B lymphocytes</p> Signup and view all the answers

    Which cells are involved in cell-mediated immunological processes such as graft rejection and defense against malignant cells?

    <p>T lymphocytes</p> Signup and view all the answers

    Which cells are part of the innate immune system and defend against infection in a nonspecific manner?

    <p>Innate immune system, including phagocytes, neutrophils</p> Signup and view all the answers

    Which cells are responsible for the synthesis of circulating humoral antibodies?

    <p>B lymphocytes</p> Signup and view all the answers

    Which cells are involved in cell-mediated immunological processes such as graft rejection and defense against malignant cells?

    <p>T lymphocytes</p> Signup and view all the answers

    Which cells are part of the innate immune system and defend against infection in a nonspecific manner?

    <p>Phagocytes</p> Signup and view all the answers

    Which protein is responsible for inhibiting serine proteases?

    <p>α2-macroglobulin</p> Signup and view all the answers

    Which of the following is true about the innate immune system?

    <p>It contains phagocytes and natural killer cells</p> Signup and view all the answers

    What is the characteristic feature of acute-phase pattern?

    <p>Increase in α1 and α2 fractions</p> Signup and view all the answers

    Which condition is associated with a diffuse broad gamma band on electrophoresis?

    <p>Chronic inflammatory disorders</p> Signup and view all the answers

    What is the most likely cause of a diffuse broad gamma band on electrophoresis in a patient with liver disease?

    <p>Increased immunoglobulin production</p> Signup and view all the answers

    Which type of gammopathy is associated with a diffuse broad gamma band on electrophoresis?

    <p>Polyclonal gammopathy</p> Signup and view all the answers

    Which of the following is the main cause of amyloidosis?

    <p>Accumulation of various insoluble fibrillar proteins</p> Signup and view all the answers

    What is the structure of fibrils in amyloidosis?

    <p>β-pleated sheet structure</p> Signup and view all the answers

    What are the two main causes of protein accumulation in amyloidosis?

    <p>Increased production of certain proteins and accumulation of mutated forms of other proteins (amyloid fibrils))</p> Signup and view all the answers

    Which type of amyloidosis is usually due to a monoclonal plasma cell disorder?

    <p>Primary amyloidosis</p> Signup and view all the answers

    What stimulates the production of serum amyloid A (SAA) in the liver?

    <p>Cytokines</p> Signup and view all the answers

    What is the protein that accumulates in primary amyloidosis?

    <p>A fragment of light chain of an immunoglobulin</p> Signup and view all the answers

    Which protein is responsible for the accumulation of degradation products in secondary amyloidosis?

    <p>Serum amyloid A (SAA)</p> Signup and view all the answers

    What are the degradation products that accumulate in secondary amyloidosis?

    <p>Serum amyloid A (SAA)</p> Signup and view all the answers

    Study Notes

    Serum and Plasma

    • Serum is the liquid portion of blood that remains after clotting, while plasma is the liquid portion of blood that remains unclotted.
    • Y proteins are synthesized in the liver.

    Blood Collection Tubes

    • Blood collection tubes are color-coded to identify the type of anticoagulant or additive present.

    Plasma Proteins

    • Electrophoresis is commonly used to analyze plasma proteins.
    • Thyroxine-binding globulin (TBG) is responsible for binding and transporting thyroid hormones.
    • Albumin is responsible for maintaining oncotic pressure, transporting hormones and vitamins, and buffering pH.
    • Albumin is not responsible for blood clotting.
    • A decreased albumin-globulin ratio in plasma is associated with liver disease, such as cirrhosis or hepatitis.

    Heme and Iron Binding

    • Hemopexin is responsible for binding free heme in plasma.
    • The total iron-binding capacity (TIBC) of plasma is approximately 300-400 μg/dL.
    • Transferrin is responsible for binding and transporting iron in plasma.
    • Iron is extremely toxic in biological systems and is always bound to proteins to prevent oxidative damage.

    Immunoglobulins

    • Immunoglobulins are produced by B cells and have two antigen-binding fragments (Fab) produced when digested by papain.
    • Immunoglobulins have a heavy chain and a light chain.
    • Heavy chains have a constant region and a variable region.

    Copper Binding

    • Ceruloplasmin carries 90% of the copper in plasma.

    Amyloidosis

    • Primary amyloidosis is usually due to a monoclonal plasma cell disorder.
    • Serum amyloid A (SAA) is produced in the liver in response to inflammation.
    • Amyloidosis is characterized by the accumulation of misfolded protein aggregates in tissues.

    α2-Macroglobulin

    • α2-Macroglobulin is produced by the liver and macrophages.
    • α2-Macroglobulin is a broad-spectrum protease inhibitor.
    • The reactive bond responsible for the biological actions of α2-macroglobulin is a thiol ester bond.

    Haptoglobin

    • Haptoglobin binds free hemoglobin in plasma, preventing its loss into the kidneys.
    • The Hb-Hp complex has a molecular mass of approximately 160 kDa.
    • Haptoglobin plays a role in the clearance of hemoglobin from the circulation.

    Menkes Disease

    • Menkes disease is a genetic disorder caused by a mutation in the ATP7A gene.
    • The characteristic feature of Menkes disease is a copper deficiency, leading to kinky hair and progressive neurodegeneration.

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    Plasma Proteins PDF

    Description

    Test your knowledge on inherited bisalbuminemia, a condition that does not have any pathological effects. Learn about the genetic polymorphism and the role of α1-Antitrypsin in inhibiting serine proteases. Discover the consequences of α1-Antitrypsin deficiency, such as excessive proteolitic activity and tissue damage in chronic bronchitis.

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