1.04 UTF-8 0 0 0 ehlers-danlos syndrom is due to defective processing of __________ 2 procollagen 1 what do thallassemia's lack 2 hemaglobin chain 1 emphysema occurs when there is a defective anti-________ 2 protease 1 classical hemaphillia is a defective factor ____ 2 VIII 1 a thrombosis can occur when there is an untimely activation of _______________ 2 prothrombin 1 what are four conceptually different regulatory mechanisms that control the activity of proteins after their biosynthesis? which one is irreversable 2 allosteric transition limited proteolysis (irreversible) association/dissacociation covalent modification 1 is the affinity of oxygen for hemoglobin increased or decreased by increasing the concentration of CO2 2 decreased 1 is the affinity of oxygen for hemoglobin increased or decreased by increasing the concentration of 2,3-BPG 2 decreased 1 the following are all essential roles of _______________ HIV replication programmed cell death exocytosis formation of beta-endorphin (analgesic) 2 proteases 1 what is the major cause of familial hypercholesterolemia 2 inherited deficiency in LDL receptor 1 what is the first thing that cardiologist grabs when some one is having a heart attack 2 TPA tissue plasminogen activator or streptokinase 1 what is the effect of heparin 2 prevent spontaneous blood coagulation by activating antithrombin III, which slows thrombin 1 a vitamin C dificiency leads to a problem in the assembly of _____________ 2 procollogen 1 lack of an enzyme in the golgi 2 I-cell disease 1 Blocked GTP hydrolysis is part of what disese? 2 Cholera 1 what disease is resultant of overproduction of an immunoglobulin 2 multiple myeloma 1 if the pH of the blood were to rise to a slightly more alkaline value, would this tend to increase or decrease the binding affinity of hemoglobin for oxygen 2 increase 1 what are the three AA in serine proteases 2 histidine aspartic acid serine 1 what prevents pancreatitis 2 pancreatic trypsin inhibitor 1 after which AA does chymotrypsin cleave 2 aromotics after phenylalanine tyrosine tryptophan 1 where does trypsin cleave 2 after basic AA arginine lysine 1 diisopropylphosphofluoridate is an effective nerve gas that is an irreversible inhibitor of ____________ 2 acetylcholinesterase 1 what converts a series of glutamate residues into gamma-carboxyglutamate 2 vit K 1 what is the function of Ca++ in coagulation 2 binds gla and forms ion bridges that bind to platelets this is why clotting is inhibbited by calcium chelators like citrate 1 how do dicoumarol, coumarin, and warfarin work 2 competitive inhibitors of vit K 1 how does aspirin works as an antithrombotic 2 irreversible inhibitor of cyclooxygenase (which activates platelets) 1 what effects does urea have on proteins 2 denatures disulfide bonds 1 what is the benefit to measuring hemoglobin A1c 2 good measure of glucose control in diabetic pts Hemoglobin A1c is a Schiff base glycosylated hemoglobin 1 does myoblobin have mostly alpha helixes or beta sheets 2 75% alpha helixes 1 is deoxyhemoglobin in the T or R configuration 2 T 1 what is hemoglobin S 2 heterozygous for sickle cell amenia resistance to malaria mutant valine residue causes aggregation 1 what is the most common protein in vertebrates 2 collagen 25% 1 What is the orientation of collagen in fibrils 2 collagen triple helixes 1 what does procollagen require for proper formation 2 Vit C hydroxyproline 1 what is required for proper cross-linkages in collagen fibrils 2 lysyl oxidase (which is copper requiring) 1 what contributes to the lack of flexibility in the elderly 2 crosslinking of collagen chains progresses with time 1 what is lathyrism 2 ingesting common sweetpea which contains beta-aminopropionitrile which inhibits lysyl oxidase cuases curvature of spine and rupture of the aorta 1 what is the MO of clostridium histolyticum 2 secretes a collagenase which increases the invasiveness of the bacterium and leads to gangrene 1 osteogenesis imperfecta is a disorder of _______________ metabolism 2 collagen 1 lysyl oxidase in involved in the formation of what two structures 2 collagen elastin 1 what is the mechanism leading to emphysema 2 smoking --> H2O2 -->oxidizes critical methionine resideu in alpha-1-antitrypsin which is an inhibitor of elastase 1 what is the cuase of Marfan's syndrome 2 genetic defect in fibrillin which makes up extracellular matrix microfibrils which is important in laying down elastin fibers and collagen fibers prone to aortic rupture (lack of elastin) 1 what do keratin filaments contribute to? 2 intermediate filaments 1 what do alpha and beta tubulin make 2 microtubules 1 which anti-cancer drugs works to inhibit microtubule assembly 2 colchicine (used to treat gout)--inhibits phagocytosis of the uric acid crystals and thus no inflammation) vinblastine vincristine podophyllotoxin 1 what anti-cancer drug inhibits the disassembly of microtubules 2 taxol 1 what will competative inhibitors do to Vmax and Km 2 increase Km no change of Vmax 1 what is the effect on Km and Vmax with non-competative inhibitors 2 decrease Vmax and do not effect Km 1 what is the target enzyme for allopurinol 2 xanthine oxidase 1 what is the target enzyme for 5-fluorouricil 2 thymidylate synthetase 1 what is the target enzyme for penicillin 2 bacterial transpeptidase 1 sulfonomides conpetively inhibit __________ incorporation into folates in baceria 2 PABA 1 what is the effect of caffeine 2 inhibitor of cyclic AMP phosphodiesterase 1 what is the rule for doses of cross-reactive drugs 2 1/10 of Km 1 what are some things that affect the activity of P450's 2 grapefruit charcoal broiled meats cigarette smoke alcohol 1 what is the role of genetic polymorphisms in relation to drug metabolism 2 may be more or less potent in pt 1 what is the idea behing Gleevec 2 rational drug design for chronic myelogenous leukemia targets aberrant tyrosine protein kinase 1 both cholera and pertussis activate ________________ by _________________ producing very large increases in 3,5, cAMP 2 adenylyl cyclase ADP-ribosylation cholera: continually active stimulator with Gs pertussis: preventing activity via Gi 1 when are caspases activated 2 apoptosis signal 1 when do we see Map kinase kinase kinase 2 carcinogensis 1 what is the benefit of liposomes 2 delivery of drugs inot the cytoplasm 1 fatty acyl and prenyl groups act as _____________ 2 hydrophobic anchors 1 most iron in mammals is bound to __________ 2 ferritin 1 where do we see peroxidation ou autoxidation 2 polyunsaturated fatty acids leading to rancidity 1 when do we see malondialdehyde 2 generated from lipid peroxidation forms lipofuscin age pigment--cause of aging 1 how do we measure free radicals 2 measure oxidation damage via pentane, ethane, and ethene in breath TBARS, Electron spin: EPR, ESR 1 how do we measure DNA oxidation 2 8-oxo-guanine accumulates in urine 1 superoxide radical and nitric oxide form ___________ 2 peroxynitrite a potent nonradical oxidant 1 _____________________ synthesizes large amounts of nitric oxide which can react with superoxide to form peroxynitrite 2 iNOS 1 excessive NMDA activity is known to produce excessive ___________ and _______ 2 nitric oxide peroxynitrite 1 what does myeloperoxidase do? 2 produces hypochlorous acid (HCLO, laudry bleach) for respiratory bursts 1 what is the function of catalases 2 degradatino of hydrogen peroxide 1 what is the major source of NADPH 2 PPP 1 favism is a deficiency in _________ 2 G6PD glucose 6 phosphate dehydrogenase (part of PPP) 1 some antimalarial drugs work by _____________ _______________ _________ 2 inducing oxidative stress 1 what is the function of SOD superoxide dismutase 2 superoxide radicals are both oxidized and reduced to generate hydrogen peroxide and molecular oxygen 1 the protein metallothioneine binds excess _____________ and lowers the toxicity of the excess and is found to have SOD activity 2 copper, zinc, and some other ions 1 phenolic antioxidants are effective because of their 2 resonance structures 1 carotenoids are particularly effective in _______________ _____________ __________ 2 scavenging singlet oxygen 1 the majority of NADH and FADH2 are produced through 2 the TCA cycle 1 at rest most of the fuel that is being used in our bodies is in the form of 2 fats 1 what vitamin is part of the NAD+ structure 2 niacin 1 what enzyme is inhibited by floride 2 enolase 1 what vitamin is incorporated into the stucture of Coenzyme A 2 pantothenic acid B2 1 what vit is incorporated into the structure of FAD 2 riboflavin B2 1 most radicals are formed during the oxydative chain becuase of what risky intermediate 2 semiquinone 1 what do we use thiosulfate for 2 antidote for cyanide poisoning 1 where does succinate enter the oxydative chain where does NADH enter the oxydative chain 2 complex II complex I 1 fotation of the gamma subunit in the ATP synthase comples is in what direction 2 counterclockwise 1 what is the function of the glycerol phosphate shuttle 2 gets electrons across the mito membrane that NAD and FAD can't cross 1 what is the function of 2,4 dinitrophenol 2 uncoupler of axidative phosphorylation 1 what are some inhibitors of complex I 2 MPTP amobarbital rotenone 1 is oxydative phosphorylation is so much more productive why do we rely so much on glycolysis 2 it is quick 1 LHON and MELAS are both examples of __________________ 2 genetic mitochondral diseases 1 what are four starvation signals 2 glucagon adenylate cyclase Protein Kinase A cAMP 1 where is Epinephrine produced and stored 2 adrenal medulla 1 strychnine, arsenic, and cyanide all poison what two enzymes 2 pyruvate dehydrogenase comples 2-ketoglutarate dehydrogenase complex 1 how are the majority of galactosemias formed 2 deficiency of galactose 1-phosphate uridylyl trnasferase 1 what is von Bierke's disease 2 glucose-6-Pase deficiency decreased gluconeogenesis leads to lactic acidosis 1 asians are often deficient in which enzyme? This causes them to get flushed at cocktail parties 2 aldehyde dehydrogenase 1