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Lesson 5: Amino Acids, Peptides, Proteins (Clinically Significant Proteins- GC Globulin, Ceruloplasmin, Haptoglobin, Beta-2-Microglobulin, Transferrin, Hemopexin, Fibrinogen, Complement, Immunoglobulin)

Terms in this set (72)

GC Globulin
It is a group specific globulin
vitamin D and actin
GC Globulin exhibits activity with ________ (a binding protein) and _______
alpha-1 and alpha-2 interzone
Where does GC Globulin migrate during electrophoresis?
radial immunodiffusion
Analytical method for GC Globulin
20-55 mg/dL
Reference value for GC Globulin
Ceruloplasmin
Alpha-2 globulin in the plasma, being the form in which most of the plasma copper is transported
Ceruloplasmin
Catalyst for redox reactions in plasma
Fe2+ to Fe3+
What does Ceruloplasmin oxidize?
membrane lipid oxidation
What does Ceruloplasmin control?
superoxide
In the presence of_________, ceruloplasmin promotes LDL oxidation, which may contribute to atherosclerosis
Ceruloplasmin
A protein that has limited role in plasma transport to tissue
Genetic deficiency, Dietary deficiency and malabsorption, Menke's disease, Wilson's Disease
What are the clinical significance decreases ceruloplasmin?
Dietary deficiency and malabsorption
(Clinical Significance - Decrease Ceruloplasmin) Lack of incorporation of copper into the molecule during the synthesis
Dietary deficiency and malabsorption
(Clinical Significance - Decrease Ceruloplasmin) Nutritional copper deficiency
Menke's Disease
X-linked inherited disorder in which dietary copper is absorbed by the gastrointestinal cells that lack an ATPase for copper transfer to plasma, hence, copper is not available to the liver for incorporation in to Cp.
Sparse, brittle and kinky hair, Growth restriction, Neurologic degeneration, Death during the first few years of life if untreated
Menke's Disease can be manifested by:
Wilson's Disease
Hepatolenticular degeneration
Wilson's Disease
Body copper is markedly increased and is deposited in tissue, including in the hepatic parenchymal cells, the brain, and the periphery of the iris.
P type
Its absence in ATPase prevents incorporation of copper into Cp
2nd or 3rd decade of life
When does manifestation of Wilson's disease happens?
Immunoturbidimetry, Immunonephelometry
Ceruloplasmin can analytical methods
proteolytic degradation
(Laboratory Consideration-Ceruloplasmin) Ceruloplasmins is subjected to ___________ during storage, that's why serum or plasma should be separated as soon as possible after collection
4C
At what temp must ceruloplasmin be stored for 3 days?
-70 C
At what temp must ceruloplasmin be stored for longer storage?
0.2-0.6 g/L
Ceruloplasmin reference value in adults
Haptoglobin
Alpha - 2 glycoprotein that binds free hemoglobin
Haptoglobin
A protein that prevents loss of hemoglobin into the urine
Radial immunodiffusion, Immunonephelometry
Analytical Methods for Haptoglobin (2)
26-185 Mg/dl
Reference range of Haptoglobin
Beta-2-Microglobulin
It is the light chain component of the major histocompatibility complex
nucleated cells
Beta-2-Microglobulin is found in the surface of what type of cells?
CD8+ cells
Haptoglobin is needed in the production of what cells?
glomerulus
Where is haptoglobin freely filtered?
proximal tubule
Where is haptoglobin reabsorbed?
Renal failure, Multiple myeloma, Rheumatoid arthritis, SLE, HIV
Elevated clinical significance of haptoglobin
Immunoassay
Analytical method for Haptoglobin
0.2-28 ug/dl
Reference value of Haptoglobin
Transferrin
A glycoprotein synthesized by the liver that transports iron to its storage sites
Transferrin
A protein that prevents iron loss through the kidneys
hemosiderin
Deficiency of Transferrin may result in the accumulation of iron into tissues which produce?
Transferrin
Major component of the beta-2 globulin fraction
Iron Deficiency Anemia
Low plasma transferrin levels can impair hemoglobin production and lead to anemia
Immunodiffusion, Immunonephelometry
Analytical Method for Transferrin
215-365 mg/dl
Reference value of Transferrin for Male
250-380 mg/dl
Reference value of Transferrin for Female
Hemopexin
Binds heme released by degradation of hemoglobin
Hemopexin
Protein that has the strongest affinity for heme
Hemopexin
Protein that helps in the diagnosis of early hemolysis
beta region
Where does Hemopexin migrate during electrophoresis
50-115 mg/dl
Reference value for Hemopexin
Fibrinogen
A coagulation factor that is converted to fibrin through the action of thrombin
Fibrinogen
Protein that forms fibrin clot when activated
Fibrinogen
Most abundant coagulation factor and one of the largest protein in the blood
Fibrinogen
Appears as a distinct band between the beta and gamma region during electrophoresis
Fibrinogen
Marker for long term prognosis of cardiovascular disease
200-400 mg/dl
reference values for Fibrinogen
Complement
One of the natural defense mechanisms that protects the human body from infection
immune cytolysis
Complement is a heat labile factor that cause _________
Complement
Protein that is circulated as nonfunctional precursors
C3
It is the most abundant form of complement in serum
Immunonephelometry, Turbidimetry
Analytical Method for Complement (2)
Immunoglobulin
A protein with known antibody activity and are the major participants in humoral immunity
plasma cells
Immunoglobulin is produced by what type of cells?
gamma globulins
Immunoglobulin's other term because they migrate to the gamma region during electrophoresis
IgG, IgA, IgM, IgD, IgE
5 SUBTYPES of Immunoglobulin
IgG
Immunoglobulin that is most abundant in plasma
IgA
Immunoglobulin that is the main antibody found in secretions
IgM
Immunoglobulin that is the first antibody to increase in response to stimulation
IgD
Immunoglobulin that is found mostly on the surface B cells
IgE
Immunoglobulin that is associated with anaphylactic reactions
Hepatic disease, Infections, Toxoplasmosis, Cytomegalovirus infections, Rubella, Herpes, Syphilis, Allergic reactions, Multiple myeloma
Immunoglobulin is increased in this disorders (9)
Nephelometry, Turbidimetry, Radial immunodiffusion, Immunoassay
4 IMMUNOGLOBULIN - ANALYTICAL METHODS
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