biochemistry module 3 buzz words

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embryo
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113462
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biochemistry module 3 buzz words
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2011-11-06 13:01:03
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purine pyrimidine metabolism
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MS 1/Mod 3: Biochemistry, buzz words
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  1. purines
    A, G, HX, X
  2. pyrimidines
    C, U, T
  3. beta glycosidic bond
    btw C'1 of sugar and N9 of purine/N1 of pyrimidine; forms nucleoside
  4. Co-A, FAD, NAD
    uses nucleotides
  5. ATP, GTP
    used by prot kinases to cov mod prot; E sources
  6. UDP-glucose
    activated intermed in glycogen and glycoprot synth
  7. cAMP, cGMP
    IC 2nd messengers of hormones
  8. ATP function
    inhibits glycolytic enz
  9. G prot
    regulated by GTP
  10. Precursors of pyrimidine synth
    Bicarb, ammonium, aspartate
  11. end products of pyrimidine synth
    • CTP: RNA synth
    • dCTP: DNA synth
    • TMP: DNA synth
  12. carboamoyl phosphate synthetase 2
    inhib by UTP
  13. orotidylate decarboxylase
    inhib by UMP, CMP
  14. CTP synthetase
    inhib by CTP
  15. precursors for purine synth
    aspartate, CO2, glycine, glutamine, N10-formyl tetrahydrofolate, R5P
  16. End products of purine synth
    • ATP, GTP: RNA synth, prot synth, energy, metabolic intermed
    • dATP, dGTP: DNA synth
  17. PRPP synthetase
    • inhib by IMP, AMP, GMP
    • act by PRPP
  18. amidophosphoribosyltransferase
    inhib by IMP, AMP, GMP
  19. IMP->XMP
    inhib by GMP
  20. IMP->adenylosuccinate
    inhib by AMP
  21. Dihydrofolate
    prod of thymidine synthetase (dTMP production) that is converted to tetrahydrofolate via dihydrofolate reductase; drug target to prevent NA synth
  22. hyperuricemia
    increased uric acid in serum (serum urate); causes precip of urate crystals
  23. arthritis
    caused by precip of urate/uric acid; gout
  24. nephropathy
    kidney damage/ failure; caused by uric acid stones in kidney (hyperuricemia); gout
  25. tophi
    • focual urate deposits that are visible
    • gout
  26. colchicine
    relieves joint pain in gout
  27. probenecide
    increases uric acid secretion from kidney; useful when hyperuricemia is due to decreased renal excretion (vs overproduction)
  28. allopurinol
    • inhibits XO; decreases uric acid production
    • increases X and HX levels (can lead to decreased PRPP levels if HGPRT intact bc inhib pathway)
  29. dopamine
    requires purine nuc for synth; def can lead to neurological problems; prob in Lesch-Nyhan disease
  30. lymphocytopenia
    loss of B and T lymphocytes (SCIDs)
  31. ADA def
    SCIDs; elevated adenosine and deoxyadenosine (toxic to lymphocytes)
  32. orotate phosphoriboysel transferase and/or orotidylate carboxylase def
    blocks UTP, CTP prod; in hereditary orotic aciduria
  33. uridine/cytidine therapy
    in hereditary orotic aciduria; inhib carbamoyl phosphate synthetase 2 (decreases orotate synth), improvement of anemia and decreased orotate secretion
  34. 5-fluorouracil (5-FU)
    inhib thymidylate synth, anti-cancer drug
  35. hydroxyrea (HU)
    inhib ribonucleotide reductase, anti-cancer drug
  36. 6-mercaptopurine (6-MP)
    inhib de novo purine biosynth, anti-cancer drug
  37. methotrexate (MTX)
    inhib dihydrofolate reductase, anti-cancer drug
  38. mycophenolic acid
    inhib inosine monophosphate dehydrogenase in prolif T/B cells; prevents graft rejection
  39. folic acid
    not synth in humans (have to get it from diet) but IS synth in bact
  40. Sulfa drugs (sulfonamides)
    comp inhib in folic acid synth; therefore we can selectively inhib purine synth in bact and not affect our own purine synth
  41. emulsion droplets
    lipase converts TAGs to emulsion droplets; increases SA:V ratio
  42. bile
    from liver; in gall bladder until we eat a meal, then it is released; bile acids are derivatives of cholesterol and act as detergents; mixes w/ FA and MAG in duodenum to form micelles (hence detergent property)
  43. chylomicron
    carries TAGs in blood until reaches delivery site
  44. TAG lipase
    • activated by adrenaline, glucagon, ACTH
    • inhibited by insulin
  45. carnitine acyltransferase 1
    RLS; FA CoA to FA carnitine to go across mitochondria
  46. carnitine
    metabolite of lysine; adds to FA CoA to carry it across mitochondrial membrane so beta oxidation can occur
  47. branched FA breakdown
    mitochondria, ER, peroxisomes
  48. Biotin
    needed in propionyl CoA carboxylase
  49. hydroden peroxide
    in v. long chain FA breakdown; acyl dehydrogenase makes hydrogen peroxide
  50. lots of acetone in breath
    in diabetics and hungry people because low blood sugar therefore low OAA and acetyl CoA build up so ketogenesis is used; but if ketone bodies build up the ketoacidosis
  51. phosphopantetheine
    in ACP; prosthetic group
  52. acetyl CoA carboxylase
    RLS of FA biosynthesis
  53. AMPK
    inactivates acetyl CoA carboxylase; inhib by ATP and act by AMP
  54. protein phosphatase 2A
    activates acetyl CoA carboxylase via dephosphorylation; inhib by PKA and act by insulin
  55. PKA
    • act by adrenaline
    • inhib by glucagon
  56. citrate
    binds allosterically to Acetyl CoA carboxylase
  57. desaturation of FA enzyme complex
    NADH->NADH cytochrome b5 reductase->cytochrome b5->desaturase->stearoyl CoA
  58. MCAD deficiency
    lesions in med chain acyl dehydrogenase (in FA oxidation); accumulation of FA such as octanoic acid
  59. Zellweger syndrome
    no peroxisomes; can't metab FA or lipids
  60. X-linked Adrenoleukodystrophy
    mut in v. long chain acyl CoA dehydrogenase; accumulation of FA such as hexanoic acid

    rx: lorenzo's oil
  61. Lorenzo's Oil
    rx for x-linked adrenoleukodystrophy

    • glyceryl trioleate
    • glyceryl trierucate
  62. pepsinogen
    pepsin; autoact when in acidic stomach env, conf change lets it digest itself...given molecule of protein acts on itself
  63. pancreatic dig enz
    serine proteases (trypsin, chymotrypsin, elastase, blood coagulation, complement factors)
  64. salt link
    in pancreatic dig enz activation; phobic N chain makes a salt link w/ Asp or Glu in AS
  65. enteropeptidase
    duodenal; cleavs trypsinogen
  66. tyrpsin
    • autocatalysis
    • cleaves panc enz prot
  67. Ser 195
    chymotrypsin, charge relay system
  68. Asp 194
    chymotrypsin; neg beta carboxyl forms salt linke w/ Ilu 16

    can be replaced by Glu; mainly conserved
  69. Gly 193
    Amid H forms H bond w/ substrate; in wrong position until activation; chymotrypsin
  70. Met 192
    chymotrypsin; side chain covers subst spec pocket and prevents subst binding

    ONLY VARIABLE AA: can be exchanged for Lys, Gln, Glu, Arg; charge and polarity obvs not important but SIZE is..want a medium sized side chain
  71. Cys 191
    chymotrypsin; disulfide keeps turning force confined ot sm area...allows gtr effect of mvmt of above residues
  72. chymotrypsinogen
    192: Met
  73. trypsinogen
    192: Gln
  74. Proelastase
    192: Asn
  75. pancreatic trypsin inhibitor
    inhibits trypsin act inappropriately
  76. alpha 1-antitrypsin
    usually has Met to fit into subst spec pocket of elastase and inhibit elastase..prevents premature activation of elastase which eats lung tissue
  77. met sulfoxide
    tobacco smoke; oxidized met; no longer fits in subst spec pocket of elastase...no inhibition and elastin of lung alveoli is eaten...emphysema
  78. procollagenase
    premature activation leads to arthritis

    bact cleave it in periodontitis and collagen degraded
  79. prekallikrein
    cleaved into kallikrein via XIIa and Hmwk in intrinsic coagulation pathway
  80. alpha macroglobulin
    traps proteolytic coagulation enz; enz cleaves alpha cov bond and results in loss of protective oligos and now BOTH enz and alpha sent to liver
  81. thioester
    alpha 2 macroglobulin, C3, C4
  82. antithrombin III
    inhibits thrombin (+) when activated by heparin (-)
  83. alpha 1 antitrypsin, TF inhib, serpin, protein Z
    inhib blood coagulation
  84. APC
    thrombin + thrombomodulin + C

    eats Va and VIIIa
  85. LPA
    • correlated w/ cholesterol intake
    • competes w/ plasminogen for fibrin binding and stabilizes clot formation
  86. vitamin K
    in gamma carboxyglutamate (adheres Ca to VII, IX, X, prothrombin, Prot C)

    similar in structure to dicumarol and warfarin drugs...inhibits coagulation
  87. Von Willebrand factor
    helps VII and platelets to interact
  88. DAF
    on membranes of RBCs; accelerates C3b + H + I to inhibit complement activation so RBCs are not destroyed
  89. paroxysmal nocturnal hemoglobinuria
    lacks DAF

    infection results in red urine at night bc path presence creates lots C3b and binds to RBCs...RBC lysis
  90. pteridine
    group in THF
  91. p-aminobenzoate
    group in THF
  92. gluatmate (carb metab)
    group in THF
  93. folate
    only in diet, makes THF
  94. sulfanilamide
    inhib folate synth via pABA
  95. ser transhydroxy methylase
    Ser-gly in N5,N10 methylene THF prod
  96. formyl THF synthase
    THF-N10 formyl THF
  97. N10 formyl THF
    • most oxidative carbon group
    • purine synth
  98. fluroruracil
    conv to flurordeoxyuridylate
  99. flurorodeoxyuridylate
    suicide inhib of thymidylate synthase
  100. aminopterin
    inhib DHFR...no THF made
  101. methotrexate
    inhib DHFR, no THF made
  102. adrenalin
    SAM acceptor
  103. creatine
    SAM acceptor
  104. melatonin
    SAM acceptor
  105. histones
    SAM acceptor
  106. NA
    SAM acceptor
  107. lipids (carb)
    SAM acceptor
  108. AA (carb)
    SAM acceptor
  109. methionine synthase
    needs biotin, homocystein->methionine, THF
  110. cobalamin
    aka B12, only made by anaerobic bact
  111. 5'-deoxyadenosyl cabalmin
    aka coenz B12, gives radical for methylmalonyl CoA mutase to make succinyl CoA
  112. hematopoeisis (carb)
    affected by B12 def; lack of THF for DNA biosynth
  113. anemia (carb)
    due to B12 def...no THF...no DNA synth
  114. corrin ring
    B12
  115. cobalt
    B12, in center
  116. 6th ligand
    B12; cyano, methyl, hydroxy, coenz B12
  117. acidosis, no myelin
    methylmalonyl CoA metab mistakes
  118. methylmalonic acid
    in urine; due to lots L-methyl malonyl CoA in methylmalonyl CoA metab mistakes
  119. alzheimers, MS
    due to B12 def...lots methylmalonyl CoA...blocks FA synth aka myelin
  120. pernicious anemai
    to due to IF in B12 def

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