Genetic Polymorphism and α1-Antitrypsin
66 Questions
5 Views

Choose a study mode

Play Quiz
Study Flashcards
Spaced Repetition
Chat to lesson

Podcast

Play an AI-generated podcast conversation about this lesson

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.

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Related Documents

    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.

    More Like This

    Serum Albumin Functions
    18 questions

    Albumin Functions Quiz and Flashcards: Learn Serum Albumin Roles

    SmoothNeodymium avatar
    SmoothNeodymium
    Plasma Cell Dyscrasias Overview
    30 questions

    Plasma Cell Dyscrasias Overview

    SufficientBohrium avatar
    SufficientBohrium
    Blood Proteins and Their Clinical Importance
    32 questions

    Blood Proteins and Their Clinical Importance

    RetractableNephrite6474 avatar
    RetractableNephrite6474
    Blood Proteins and Clinical Importance
    32 questions

    Blood Proteins and Clinical Importance

    RetractableNephrite6474 avatar
    RetractableNephrite6474
    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser