Plasma Proteins Flashcards
Only plasma proteins not synthesized in the liver.
Gamma globulins: made in plasma cells (IgGs)
also clotting Factor VIII
Major protein responsible for osmotic pressure in the blood.
Albumin
What two things does Transthyretin (prealbumin) bind?
- Retinol
2. Thyroxine (T4)
Function of Alpha-1-antitrypsin and defect that occurs when a deficiency is present.
Inhibits serum proteases (mainly elastase)
Deficiency results in alveolar destruction and emphysema.
Name 3 instances where alpha-fetoprotein increases.
- In maternal blood when a fetus may have a body wall defect
- In patients with hepatocellular carcinoma
- In patients with a testicular teratoma
Function of alpha-2 macroglobulin
Protease inhibitor
Transports Zinc
Binds cytokines
Function of Haptoglobin.
Binds Hb and prevents loss during hemolysis.
Function of Hemopexin.
Binds heme and hematin to consever iron
Function of transferrin.
Transports iron in plasma. Measured to determine a patients total iron binding capacity.
Who are bronze diabetics?
Patients with hemochromatosis
- HFE gene mutation leading to loss of regulation of GI iron absorption, normally high iron levels act as a negative feedback to prevent absorption of iron in the small intestines but his mechanism does not work
- fatal if untreated due to liver disease, heart failure, diabetes
Tx for hemochromatosis.
repeated phlebotomies
Pathology of Wilson Disease.
Mutation in hepatic Copper-binding ATPase
- causes defective excretion of copper into the bile
- reduced attachment to ceruloplasmin
- accumulation of copper in liver, brain, and kidney
Tx for Wilson Disease.
Penicillamine
Pathology of Menke Disease
Mutation in extra-hepatic Copper-binding ATPase
-low Copper in plasma causing reduced activity of some essential enzymes
Major acute phase protein released as a result of trauma or inflammation.
C-reactive Protein
gamma globulin
