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Biochemistry Flashcards

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8541523740coagulation factors I and Vwhat are the coagulation factors released on platelet activation0
8541523680erythropoietin - kidneywhat regulates RBC production - where is it released from?1
8541523681O2 levelswhat is the most important regulator of RBC production?2
8541523682low O2what level, high or low, of O2 stimulates RBC production through the action of erythropoietin3
8541523683low blood volume, anemia, low Hb, poor blood flow, pulmonary disease, altitudewhat are some things that decrease O2 levels, thus causing increased RBC production4
8541523684increased RBC productionwhat is polycthemia5
8541523685too little O2 in the airwhat is physiologic polycythemia6
8541523686mutation that doesn't stop RBC production when there is enough - therefore there are too many RBC made patients have reddened appearance of the skinwhat is polycythemia vera7
8541523687vitamin B12, folic acid, intrinsic factor (allows B12 to cross ileum)what vitamins are necessary for RBC production8
8541523688pernicious anemia -failure to absorb vitamin B12 from the GI tractlack in intrinsic factor is called what?9
8541523689stimulates release of oxygen (to the left)how does 2,3-BPG shift the Hb curve?10
8541523690spleenwhere do RBC self-destruct?11
8541523691defective synthesis of membrane cytoskeletal proteins and makes the RBC susceptible to mechanical stress - cells become sphericalwhat is spherocytosis12
8541523692glucosewhat does the RBC use as energy?13
8541523693lactate dehydrogenasewhat enzyme in the RBC converts pyruvate into lactate?14
8541523694glycolysiswhat is the only metabolic pathway in RBCs?15
8541523695pentose phosphate pathwayG6PD is important in what offshoot pathway in the RBC?16
8541523696methemoglobinwhat is another name for Fe3+?17
8541523697caused by ROSwhat causes methemoglobin?18
8541523698decreased O2 deliverywhat is bad about methemoglobin19
8541523699NADHwhat is used to regenerate Fe2+ from Fe3+?20
8541523700increases O2 delivery to tissueswhat does the 2,3-BPG shunt do?21
8541523701decreased ATP production -RBC ion transporters are affected and this causes hemolytic anemia -can be masked by effects of 2,3BPGwhat happens in pyruvate kinase deficiency?22
8541523702gain Ca, lose K, lose H2Owhat changes in Ca, K, H2O in pyruvate kinase deficiency?23
8541523703cannot protect membrane proteins against oxidationwhat happens in G6PD deficiency?24
8541523704RBC have shorter lifespanwhat happens to RBC in G6PD deficiency?25
8541523705more resistant to malariawhat is one "benefit" of G6PD deficiency?26
8541523706ironwhat ion is required for heme synthesis?27
8541523707transferrinwhat protein does iron bind with to be transported throughout the body?28
8541523708ferritinwhat is the name of the iron storage molecule in the liver?29
8541523709complex of ferritin moleculeswhat is hemosiderin30
8541523710microcytic and hypochromic anemiawhat is the anemia in iron deficiency anemia?31
8541523711iron deficiency anemia32
8541523712bilirubinthe porphyrin part of Hb is converted into what?33
8541523713high - patient often presents with jaundicein hemolytic anemia, what are the bilirubin levels like?34
8541523714hemolytic anemia35
8541523715porphyriaswhat are deficiencies of any enzyme in the heme synthesis pathway called?36
8541523716gamma-aminolevulinic acid synthasewhat is the main regulatory enzyme of the heme synthesis pathway37
8541523717heme (decreases enzyme activity)gamma-aminolevulinic acid synthase is regulated by what molecule? what change?38
8541523718uroporphyrinogen decarboxylaseporphyria cutanea tarda is a deficiency in what enzyme?39
8541523719scarring and increased facial hair growthporphyria cutanea tarda has what s/s40
8541523720gamma is swapped for betapost-natal, what is the change in globulin chains?41
8541523721large excess of 1 subunit of Hbwhat are the thalassemias42
8541523722beta chain deficiencywhat is the most severe thalassemia?43
8541523723heterozygouswhat type of thalassemia is more resistant to malaria?44
8541523724Glu6Val on β-globinmost common mutation causing sickle cell disease45
8541523725more likely to aggregate in deoxygenated statewhen are HbS (sickle cell anemia) most likely to aggregate?46
8541523726heterozygous formwhat form of sickle cell disease is more resistant to malaria?47
8541523727increase EPO releasehow does anemia increase RBC production48
85415237285 dayshow long does it take to see an increase in RBC/increase in hematocrit?49
8541523729impaired Hb synthesismicrocytic, hypochromic anemia - cause?50
8541523730red cell lossnormocytic, normochromic anemia - cause?51
8541523731macrocytic, normochromic anemia (megaloblastic anemia)what type of red cell morphology anemias do vitamin B12 or folic acid deficiencies cause?52
8541523732O - bc have no antigens (cannot be attacked by host cell)Which blood type is aka the "universal donor" and why?53
8541523733AB - bc it doesn't produce Ab that attack incoming bloodWhich could be called the "universal acceptor" and why?54
8541523734A+ or OWhat type(s) of blood can someone with A+ blood receive in a transfusion?55
8541523735dad is Rh+ and mom is Rh- and baby is Rh+...but this happens after the moms first kid because mom created Rh factor bc of exposure to kid's Rh+erythroblastosis fetalis happens with what combination of Rh?56
8541523736rhogamwhat drug is used to prevent erythroblastosis fetalis?57
8541523737Rh+ RBCs in fetus attacked by mother's antibodieswhat happens in erythroblastosis fetalis?58
8541523741promotes vasoconstrictionwhat does 5-OH tryptamine (serotonin) do when released on platelet activation59
8541523742stimulates repair of damaged tissueswhat does platelet-derived growth factor do?60
8541523743aspirinwhat inhibits the production of thromboxin A261
8541523744plavix (clopidogrel) - blocks the effects of ADPwhat is one name for an ADP receptor antagonist?62
8541523745pleckstrinwhat molecule, activated by PKC, causes aggregation and release of contents of platelet granules?63
8541523746stimulation of the polyphosphoinositide pathwayhow are prostacyclin, collagen, TxA2, thrombin, and ADP activated (in the platelet activation pathway)?64
8541523747intrinsic pathway (contact factor) extrinsic factor (tissue factor)what are the two pathways for clot formation65
8541523748contact factorwhat factor in the intrinsic clotting pathway66
8541523749tissue factorwhat factor in the extrinsic clotting pathway67
8541523750-formation of prothrombin -prothrombin to thrombin (by factor X) -conversion of fibrinogen to fibrin (by thrombin) -forms insoluble mesh -cross-linking of polymerspathway of clot formation from prothrombin --> clot68
8541523751glycoproteins - synthesized in the liver circulate in the plasmawhat are clotting factors (type of molecule), where are they made, and where do they go?69
8541523752Christmas factorwhat is factor IX also called?70
8541523753XII, XI, IX, VII, X, and prothrombinwhat factors are serine proteases and are activated in a cascade71
8541523754factors V, VIII, IIIwhat factors are cofactors that bind to other factors?72
8541523755cross-linking of fibrin monomerswhat process forms the "hard" clot?73
8541523756free (NOT fibrin bound) plasmin inactivates itwhat does alpha-2 antiplasmin bind to? and what does it do?74
8541523757plasminwhat degrades the clot?75
8541523758tissue plasminogen activator (released from vascular endothelium)what activates plasminogen (bound to fibrin in the clot) to create plasmin that eventually breaks down fibrin?76
8541523759antithrombin III (MOST IMPORTANT) alpha-1 antiprotease alpha-2 macroglobulinwhat are the three molecules that inactive thrombin that is NOT localized to the clot and prevent inappropriate clotting?77
8541523760antithrombin IIIwhat is the most important plasma inhibitor of thrombin (not localized to the clot)78
8541523761heparinwhat activates antithrombin III?79
8541523762binds thrombin and activates protein C (makes thrombin change from a procoagulant to an anticoagulant at sites where clots shouldn't form)what does thrombomodulin do?80
8541523763activates antithrombin III to prevent inappropriate clottingwhat does heparan sulphate do?81
8541523764inhibits platelet activationwhat does prostacyclin do?82
8541523765becomes anticoagulant after binding thrombomodulin on the cell surfaceafter binding thrombomodulin, what happens to thrombin?83
8541523766DVTmutations in proteins C and S can cause what?84
8541523767protein Swhat is the cofactor for protein C?85
8541523768V and VIIIwhen thrombin becomes an anticoagulant, what factors does it work on? to prevent clotting?86
8541523769X Linked recessiveinheritance pattern of hemophilia A87
8541523770deficiency of factor VIII (intrinsic pathway)what is the deficiency in hemophilia A88
8541523771spontaneous bleeding into joints and soft tissueswhat happens in hemophilia A89
8541523772replacement therapy of factor VIIItreatment of hemophilia A90
8541523773x linked recessiveinheritance pattern of hemophilia B91
8541523774deficiency of factor IX (intrinsic pathway)what is the deficiency in hemophilia B92
8541523775replacement therapytreatment of hemophilia B93
8541523776autosomal dominantinheritance pattern of hemophilia C94
8541523777deficiency in factor XIdeficiency in what for hemophilia C95
8541523778autosomal dominantvon willebrand disease96
8541523779deficiency of von willebrand factordeficiency in what for von willebrand disease97
8541523780reduced platelet recruitment and reduced delivery of factor VIII (which is stabilized by binding to vWF)what happens in vWB disease?98
8541523781desmopressin to promote vWF levels and replacement therapy of vWF and VIIItreatment of vWB disease99
8541523782factor Vleiden mutation is a mutation in what factor?100
8541523783caucasian europeanpopulation commonly affected with leiden mutation101
8541523784factor V is resistant to inactivation by protein S/C complex which increases the risk of DVTwhat does the mutation in leiden mutation cause?102
8541523785-maintenance of plasma pressure -binding and transport of small molecules and ions -defense against infection -blood clotting -inhibition of proteolytic enzymesfunctions of plasma proteins103
8541523786albumin (most common), globulins (a1, a2, b, y), clotting factorswhat are the classifications of plasma proteins104
8541523787liverwhere is albumin synthesized?105
8541523788liverwhere are alpha and beta globulins synthesized?106
8541523789liverwhere are clotting factors synthesized?107
8541523790plasma cellswhere are gamma globulins synthesized?108
8541523791albuminwhat is the major protein in human plasma109
8541523792albuminwhat is responsible for the osmotic pressure of plasma110
8541523793decreasesthe serum albumin level __________ in edema111
8541523794albuminwhat plasma protein is important when considering drug dosing?112
8541523795transports retinol and thyroxine (T4) - important because retinol is small enough for excretion so this saves it from excretion another name - prealbuminwhat does transthyretin transport? and what is another name for it?113
8541523796inhibits serum proteases and protects degeneration of alveolar tissuealpha-1-antitrypsin does what?114
8541523797emphysemadeficiency of A1AT causes what?115
8541523798fetal liver - small amounts in adult bloodwhere is alpha-fetoprotein made?116
8541523799hepatocellular carcinoma and testicular teratomaalpha-fetoprotein increases in what two diseases?117
8541523800fetal body wall defectsalpha-FP is used as a marker for what?118
8541523801protease inhibitor, transports Zn2+, binds cytokineswhat does alpha-2-macroglobulin do?119
8541523802haptoglobinwhat is the major component of alpha-2 globulin fraction?120
8541523803haptoglobinwhat binds hemoglobin released during hemolysis121
8541523804haptoglobinwhat prevents loss of free hemoglobin into the kidney and conserves iron122
8541523805hemolytic anemiahaptoglobin levels are depleted in what disease?123
8541523806binds heme and hematin and conserves iron beta 1-globulinwhat does hemopexin do? and what is it?124
8541523807HB released from RBC Hb divides into dimers and the dimers are picked up by haptoglobin OR the dimers break down even further and they are bound to hemopexinhow do haptoglobin and hemopexin conserve iron?125
8541523808transports iron in plasmawhat does transferrin do?126
8541523809total iron binding capcityplasma transferrin concentration is measured by what?127
8541523810ferritin appears in plasmawhen there is excess iron, what do you see?128
8541523811Hb and Mbwhere is most iron in the body found?129
8541523812autosomal recessiveinheritance pattern of hemochromatosis130
8541523813defective HFE gene on chromosome 6what and where is the defect in primary hemochromatosis131
8541523814-binds transferrin receptor and decreases its affinity for transferrin -upregulates the expression of hepcidin (a peptide that down-regulates iron release and inhibits iron absorption)what does the HFE protein NORMALLY do?132
8541523815bronze diabetes or the celtic curseother names for hemochromatosis133
8541523816--high blood iron levels because there is loss of regulation of absorption of iron in the small intestine --HFE gene (think High, FE (iron) levels)what is the major problem seen in hemochromatosis - how can you remember the gene134
8541523817binds plasma copperwhat does ceruloplasmin do?135
8541523818mutation in gene encoding hepatic Cu-binding ATPase so there is defective excretion of copper into the bile with accumulation of copper in liver, brain, and kindneywhat is wilson's disease136
8541523819penicillaminetreatment of wilson's disease137
8541523820xlinked recessive defect in Cu binding ATP ase causes fatality in childhoodwhat is menkes disease138
8541523821wilson diseasekaiser-fleischer ring in what disease?139
8541523822increase in gamma globulin bandwhat change in electrophoresis of plasma proteins in chronic conditions140
8541523823increased alpha 1, 2, and beta 2 bandswhat change in electrophoresis of plasma proteins in inflammation or after trauma141
8541523824gamma globulin bandwhat change in electrophoresis of plasma proteins in monoclonal gammopathy (like multiple myeloma)142

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