Slide 1Slide 2Plasma - blood treated with anticoagulants to prevent clot formation then centrifuged to remove the cells Serum - blood that is allowed to form a fibrin clot then centrifuged to remove the cells and the clotSlide 4Slide 5Slide 6Slide 7Slide 8Slide 9Slide 10Slide 11Slide 12Regulation of colloidal pressureSlide 14Slide 15CERULOPLASMINSlide 17Slide 18Slide 19Slide 20Blood: Cells and Plasma ProteinsBeth A. BouchardBIOC 212: Biochemistry of Human DiseaseSpring 2005I. Major functions of blood and its components: - transport and distribution of essential nutrients to tissues - removal of waste products - defense against internal insult - repair of damaged tissuesII. Two major components of blood: liquid phase and formed elementsPlasma - blood treated with anticoagulants to prevent clot formation then centrifuged to remove the cellsSerum - blood that is allowed to form a fibrin clot then centrifuged to remove the cells and the clotPlasma“Buffy coat”Red blood cellsPlasmaBuffy coatRed blood cellsRed Blood Cells(Erythrocytes)- 1011 produced per day- Anucleate- Lack intracellular organelles (incapable of protein synthesis and repair)- Contain specific proteins and ions- Transport oxygen, and remove CO2 and H+- Finite life span of 60-120 days beforebeing destroyed in the spleen- 2-3 m in diameter- 150-400 x 106/mL blood- Normal subcellular structures except they lack a nucleus- Capable of mitochondrial protein synthesis- Platelet function is dependent upon their activation- Involved in blood clotting- Contain secretory organelles called alpha-granules, which contain proteins important in blood clotting- Circulate for 10 days before being destroyedPlateletsPlasma proteinsSynthesized by liver or produced by plasma (B) cellsA. AlbuminB. TransferrinC. CeruloplasminD. Enzymes – coagulation enzymes, complement factorsE. C-reactive protein – acute phase reactantF. Immunoglobulins – humoral immunityTransport proteins66 kDaALBUMIN-Accounts for ~50% of the total plasma protein and ~ 50% of the total liver protein production- Half-life ~ 20 days - Highly polar- At pH 7.4 it is anionic with 20 negative charges per molecule- Transport of long chain fatty acids & sterols- Transport of bilirubin- Binding and solubilization of drugsFunctions of AlbuminRegulation of colloidal pressure Decreases in albumin concentration cause edemaGaw: Clinical Biochemistry; Churchill Livingstone (1999), p. 44.Causes of decreased plasma albumin:I. Decreased synthesisA. malnutrtionB. malabsorptionC. advanced chronic liver diseaseII. Abnormal distribution or dilutionA. overhydrationB. increased capillary permeability like in septicemiaIII. Abnormal excretion or degradationA. nephrotic syndromeB. burnsC. hemorrhageD. certain catabolic statesE. protein losing enteropathies IV. Rare congenital defects A. hypoalbuminemia B. analbuminemiaTRANSFERRIN- Transports iron in plasma as ferric ions (Fe3+) – each transferrin molecule binds 2 Fe3+ - Protects the body against the toxic effects of free iron-Normally 30% saturated with Fe3+ - increased or decreased saturation is indicative of iron overload or deficiency, respectively - Decreased in inflammatory states due to excessive degradation of transferrin- Fe3+ complexesCERULOPLASMIN-Major copper transport protein- Regulates oxidation-reduction, transport and utilization of iron- Increased concentrations are found in active liver disease or tissue damage- Decreases are sometimes observed in individuals with Wilson’s diseaseENZYMES- Enzymes of the coagulation and complement cascade have a defined function in blood- Others reside elsewhere in the body and appear incidentally in the blood – their measurement is of diagnostic valueAcid phosphatase AmylaseCreatine kinaseAlanine aminotransferaseC-REACTIVE PROTEIN (CRP)- Major component of the acute phase response and a marker of bacterial infection- Mediates the binding of foreign polysaccharides, phospholipids and complex polyanions, as well as the activation of complement- <1 mg/mL in normal plasma- Slightly elevated levels of CRP are indicative of chronic, low-grade inflammation and have been correlated with an increased risk of cardiovascular diseaseIMMUNOGLOBULINS- Proteins produced and secreted by B cells specific for the foreign particle that stimulated their synthesis- Five classes of Ig- Multiple myeloma: arises from the aberrant proliferation of a single B cell. Light chains of Igs are produced in excess of heavy chains = paraproteins. Elevated serum protein levels are usually due to an increase in the Ig fraction and may indicate the presence of a