Biochem Heme Mini2

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Biochem Heme Mini2
2011-10-29 21:22:42
Biochem Heme Mini2

Biochem Heme Mini2
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  1. Asialoglycoprotein
    • Protein tagged for Hepatocyte denaturing by removal of surface sialic acid by neuraminidase
    • Kuffer cells recognize asialoglycoproteins and engulf to destroy
  2. Alpha 1- antiTrypsin
    • Protease inhibitor (anti-coagulation, anti thrombin)
    • low levels in ZZ-homo deficiency -- pulmonary emphysema resulting from overactive protease
    • Low level also a sign of hepatic transport disorder due to liver damage (trypsin inhibitor overstorage)
  3. Alpha 1-Macroglobulin
    • Protease inhibitor (anti-coagulation AND anti-clot breaker)
    • -- inhibits both thrombin (clotting) and plasmin (fibrinolysis)
    • Onocotic pressure keeper in nephrotic patients (replaces albulmin)
    • Elevation indicaties renal failure or analbuminenia as proteins are peed out
  4. Beta 1 band on protein electrophorisis
    • Apoprotein Transferrin - transport Ferric Iron (3+)
    • Increase in transferrin (TIBC) - is a sign of iron deficiency
  5. Beta 2 band on protein eletrophorisis
    • Fibrinogen clotting factor
    • C3 compliment protein
    • some reason has to with obstructive jaundice
  6. Albumin (most neg charged on electrophorisis)
    • Unconjugated Bilirubin transport (from spleen to liver)
    • Oncotic pressure
    • drug transport (warfrin and penicillin)
    • Decrease means nephotic syndrom, or tissue inflammation or blood loss (could be genetic as well)
  7. Thyroxine
    Thyroid hormones T3/T4 transport
  8. Retinol binding protein
    Vita A transport
  9. Haptoglobin
    • Free hemoglobin transport
    • Scavenges hemoglobin diners from blood
    • Used for intravescular hemolysis indicator (DIC would cause decrease in Hpt)
    • Destroyed along with heme it transports
  10. Hemopexin
    • Heme that escapes spleen and liver degradation is scavenged by hemopexin
    • decrease Hpx could also be hemolytic anemia
  11. Ceruloplasmin
    • Copper transport
    • absence results in wilson's disease and copper deposits in eye (kaiser fletcher ring)
    • Transferrin
  12. Alpha fetoprotein
    • increase in prego means neuro tube defect
    • decrease in prego means down's syndrom
    • increase in adult means liver cancer (cause the fuck are you doing producing a fetal protein liver...)
  13. C-reactive protein
    acute infection
  14. Liver enzymes
    • Liver makes a lot of enzymes
    • --Choline esterases
    • -- Transaminases (AST/ALT)
    • -- Phosphotases (ALP/AP)
    • -- GGT
    • -- LDH
    • -- CK
  15. Choline-esterase
    • degrades choline (neurotransmittor)
    • Decrease in choline esterase:
    • --overdose of choline esterase inhibitor (Myathenia gravis therapy)
    • --Organophosphate poisoning
    • --Scoline apnea (succinylcholine esterase deficiency)
    • --Liver failure (it no make)

    • Increase in choline esterase:
    • --nephrotic syndrom (i pee it out)
    • -- liver tumor (it make too much)
  16. SuccinylCholine
    • Anasthetic
    • Genetic Scoline apnea has deficient coline esterase for succinlcholine
    • Over activation of anasthetic effects
  17. AST
    • Aspartate transaminase
    • -- Increase ALONE - MI/skeleto cell damage or RBC lysis
    • -- Increase WITH ALT - acute liver hepatitus
    • Normal increase with neonatal hemolysis due to RBC turnover
  18. ALT
    • Alanin transaminase
    • --increase ALONE - liver cirrhosis/disease
    • --Increase WITH AST - acute liver hepatitus
  19. ALP
    • Alkaline Phosphotase- released by hypertrophied cartilage to lay down calcium
    • --Increase in ADULT WITH GGT means bile duct damage
    • --Increase in ADULT WITHOUT GGT means bone tumor or some shit
    • --Increase in CHILDHOOD/Placenta/After meals normal due to increase in bone formation and bile duct normal function
    • use gammaGT to follow up billiary duct damage
  20. gammaGT
    • Billiary protein
    • Increase means cholestatic liver Disease
    • useful over AST < ALT < ALP (from least useful to most besides gamma GT)
    • Also a sign of alcohol or drug abuse
  21. LDH
    • Lactate dehydrogenase
    • increase in LDH1 - myocardio infarction
    • increase in LDH5 - acute hepatitus
  22. Creatine Kinase
    • CK-BB for brain tumors
    • CK-MB for Myocardio infarctions
    • CK-MM for muscle dystrophies
  23. Amylase
    • mainly in pancrease
    • acute pancreatitus - you are going to get neutrophilia as well.... GOOO NEUTROPHILS
  24. Asprin as anti-clot
    • COX-1 inhibitor
    • Thromboxane inhibitor (prostacylin inhibitor too but thromboxane more preferential)
    • Will increase bleeding time test
  25. Thromboxane
    • clotting factor
    • increase platelet aggregation and number
  26. von Willibrand Factor
    • present on endothelium(bound to factor 8 to inactivate it), platelet surface, plasma soluble protein
    • binds to GP1b receptors on other platelets, promote platelet aggregation
    • Deficiency is von Wilibrand disease
    • --increase bleeding time test
  27. Thrombin (2a)
    • major clotting factor
    • intrinsic and extrinsic final effector
    • self promote by activating 8a(tenase complex) and 5a(Prothrombin complex) and 11a (christmas activator)
    • fibrin formation by cutting fibrinogen into fibrin and 13a(fibrin crosslinker) activation
  28. 9/9a
    • Christmast factor - forms 10ase complex with 8a and Ca2+
    • binds platelet with vita K carboxylation
    • Hemophelia B missing
    • can be activated by extrinsic 7a
  29. 8/8a
    • Bound to von Wilibrand when inactive
    • forms 10ase complex to activate 10a
    • hemophilia A when deficient
  30. 10ase
    • intrisinc
    • 8a+9a---platelet
    • with ca2+ turns 10agolden
  31. Extrinsic 10a
    • 7a bound platelet and tissue factor 3
    • this is tested in PT test (no intrinsic activation)
  32. Heprin
    • AntiThrombin III activator
    • kills 12a, 11a, 9a, 10a, 2a and probably 7a
    • not degraded
  33. Warfrin
    Anti-vita K, it no add COO- to factors 7, 9, 10, 2
  34. Vita K
    • Adds COO- to 7, 9, 10, 2
    • needed for blood clot
    • babies sometimes have a deficiency
  35. 12/12a
    • first one of the intrinsic (don't need cause 2a can activate 11a too)
    • pre-kallikren + HMWK+ collagen activates
  36. 13/13a
    • transglutamase
    • cross links fibrin dimers
    • activated by thrombin
  37. Fibrin
    • Dimers from inactive Fibrinogen
    • activated by Thrombin
    • forms dimers when activated and linked by 13a
  38. Prothrombin Complex
    • 10a+5a+2+Calcium
    • 10a from tenase complex
    • 5a is activated by 2a (or mechanical stress as shown by research paper)
  39. Plasmin
    • Cuts fibrin (clot buster)
    • activated by tissue plasminogen activator and urokinase
    • deactivated by alpha2 antiplasmin and plasminogen activator type 1/2
  40. Urokinase/Tissue plasminogen activator
    • plasmin activator
    • bust a clot
  41. Serpin/alpha-2 antiplasmin/plasminogen activator 1/2
    • anti-plasmin
    • don't bust a clot
    • alpha 2 macroglobulin also a anti plasmin
  42. Protein C/S
    • Thrombinmodulin activated effector
    • busts up 8a and 5a turning then back to 5/8
    • effectivly bust up tenase and prothrombin complex respectivly
  43. Thrombinmodulin
    • needs thrombin to work
    • -- an example of self regulation, lots of thrombin activate thrombinmodulin
    • activates protein C,S
    • deactivate 5a and 8a
  44. Prothrombin time (PT)
    • really checking for common pathway and extrinsic pathway
    • Tissue factor 3 is added without collagenase, only extrinsic and common is activated
    • Increased time in --vitamin K deficiency
    • --factor 7 deficiency - over comsumption of substrates in DIC
    • --warfrin therapy
  45. Partial Thromboplastin time (PTT)
    • Add in kaolin to simulate collagen
    • tests for intrinsic and common pathway, especially hemophilia A/B and von Wilibrand pathway (factor 8)
  46. Bleeding time
    • adds nothing, tests for platelet number and functionality
    • Increased time when taking asprin
    • or when there is a decresae in von Wilibrand factor (reduced platelet to platelet adhesions)
  47. DMT1
    • Transports ferrous iron into lumen cell
    • controlled by hepcidin
  48. Ferritin
    • Iron storage in cell, ferric iron
    • forms hemosiderin during iron overload
    • plasma levels correspond to bone marrow levels
    • decreased plasma levels means iron deficiency
  49. Ferriportin
    • transports ferrous iron out of cell and into plasma
    • hepcidin controlled (IL-6 indrectly controled)
  50. Transferrin
    • transports ferric iron within plasma from asportion to area of utilization
    • High capacity and low saturation indicates anemia (due to iron deficiency)
  51. Iron overload
    • thalassemia
    • genetic hemochromatosis DMT1 upregulation
    • Lead poisoning
  52. Colbalamine
    B12 with cobolt co-factor
  53. B12 absoption
    • ilium absorption requring intrinsic factor (from parietal cells)
    • lack of absorption is pernicious anemia
  54. B12 reactions
    • Methymalonyl CoA Mutase -- B12 only
    • Homoysteine methyltransferase -- WITH folate
    • if abscent --> methylmalonic acidimeia (conclusive for B12 def)
    • increased homosytein could mean B12 deficiency but also could be folate deficiency
  55. B12 Def.
    • Methymalonic acidimeia (definitive)
    • all folate anemia signs PLUS neurological disorders
  56. Folate reactions
    • makes purines and thymidine
    • mythylation of DNA/RNA
    • needed for cell replication
    • which is why cells can't divid (they grow but can't divid) without folate
    • and why pregos need more folatic acid
  57. B6 defi
    • not really part of the lecture but B6 is ALAsynthase cofactor
    • Ala synthase deficient means no Heme and produce sideroblast anemia
    • no heme/hemoglobin == siderobplast, microcytic, iron overload anemia... pretty much
  58. Folate drugs
    Leuvencore... i think, dude look at pharmaco
  59. RBC breakdown
    • Heme-->biliverdin-->bilirubin (this is in spleen)
    • bilirubin +albumin = unconjugated indirect bilirubin (this is in plasma right out of spleen)
    • bilirubin -albumin + glucuronic acid = conjudated direct bilirubin-diglucuronide (this is in hepatocyte)
    • direct bilirubin --> bile --> gut --> poop or reuptake
  60. NADPH nicotinamide cofactor
    • strong anti-oxident and produce steroids
    • pentose phosphate pathway made by G6PDH
    • shortage of NADPH means excess H2O2
    • this will be the way RBCs lyse and aqumulate heinz bodies (heme fragments) in G6PDH def
  61. G6PDH def.
    • can't lead with reactive O2 because NADPH not made from the pentose phosphate pathway
    • jaundice resulting from hemolysis
    • Heniz body (oxidative stress) Hb degraded hemolytic anemia
  62. Methemoglobin
    • Hemoglobin bound to ferric iron without oxygen
    • none functional, results from oxidative compound poisoning
    • Methemoglobin reductase to reverse
  63. Congenital Methemoglobin
    Congenital His-Try mutation that favors oxidation of ferrous iron to ferric once bound to hemoglobin
  64. Fetal Hemoglobin
    • alpha/gamma hemoglobin
    • high affinity for O2 low affinity for BPG
    • can't form salt group with BPG so decrease affinity for its negative allosteric regulator (BPG)
    • His --> Ser
    • Will take O2 from Hb (from mother) like Myoglobin
  65. CO poisoning
    • CO high affinity for Hemoglobin 25k times
    • Irreversible
    • 0.1% CO is lethal
  66. Myoglobin
    • High affinity for O2
    • takes from Hemoglobin
    • gives to cytochrome oxidase in ETC
    • O2 storage in muscle
  67. Co-operative binidng
    • Hemoglobin trait
    • Each successive O2 addition increase affinity for next O2 and O2 is scavenged
    • Each successive O2 subtraction decreases affinity and all O2 is completely released
    • Low [O2] low affinity --> release all O2
    • High [O2] high affinith --> bind all O2
  68. Negative allosteric regulators of hemoglobin
    • neg allosterics help decrease O2 affinity thus release at site of need
    • all are metabolic by products
    • BPG>CO2>Low pH
    • stablize T(deoxy state)
  69. CO2 transport
    • Hemoglobin trasports small fraction in carbamate
    • mostly as HCO3- (CO2+H2O-->H2CO3-->HCO3-) in tissue rich in CO2
    • Hb will buffer extra H+
    • Once at lung low CO2 drive reaction in reverse and HCO3- is turned back into CO2 and H2O
    • Cl- used to balance ion concentration
  70. Carbonic Anhydrase
    • Perfect enzyme, completely converts
    • CO2+H2O --> H2CO3 and reverse
    • depending on concetration