biochemistry buzz words

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  1. pyrrole
    5 mem ring, 4C, 1N
  2. porphyrinogen
    ring of 4 pyrrole joined by methylene bridges
  3. porphyrin
    ring of porphyrinogen joined via methenyl bridges
  4. pyridoxal phosphate
    req by ALA synthase (RLS)
  5. uroporphyrinogen 1
  6. ALA 2
    erythroid heme synth
  7. ALA 1
    all other cell heme synth
  8. heme and prot kinase
    when lots of heme around, heme blocks prot kinase act so that eIF2 stays active and increases globin synth

    eventually excess heme tied up with new increases in globin synth

    erythroid cells only
  9. non erythroid heme regulation
    heme inhib ALA 1 transport, synth, and act
  10. reticuloendothelial system
    in macrophages, monocytes, kupffer cells

    heme degradation system using hemeoxygenase
  11. heme oxygenase stimulation
    via heme, tin , cobalt, stress
  12. porphyrias
    lesions in heme synth
  13. X linked anemia
    lesion in ALA synthase

    decreased heme and therefore hemoglobin
  14. aminolevulinic acid dehydratase porphyria
    build up of delta-aminolevolunic

    lead poisoning
  15. acute intermitten porphyria
    increased porphobilinogen and delta aminolevulinic (steps 1 and 2 prob)
  16. congenital erythropoietic porphyria
    increased uroporphyrinogen 1, uroporphyrin 1, coproporphyrinogen 1, coproporphyrin 1
  17. porphyria cutanea tarda
    • increased uroporphyrin 3 (step 4)
    • induced by hexachlorobenzene
    • rx: chloroquin, phlebotomy
  18. hereditary coproporphyria
    increased coproporphyrin 3
  19. variegate porphyria
    increased porphyrins
  20. erythropoietic protoporphyria
    lots porphyrins in skin
  21. lead poisoning
    has symptoms of porphyria bc delta aminolevulinic acid dehydratase and ferrochetalase are inhib by lead due to their sulfhydryl groups (first and last steps in heme synth)
  22. ab porphyria etiology
    toxic intermed in liver...pain
  23. neurological poryphria etiology
    toxic intermed in brain
  24. skin porphyrai etiology
    toxic stuff in skin conv to free radicals under UV radiation
  25. teeth porphyria etiology
    toxic stuff causes discoloration and brittleness
  26. phenobarbital
    tells liver to make p450 (has heme group)...therefore lots hemes made and intermed accum
  27. sex hormones and porphyria
    sx not until puberty bc inc sex hormones can contain p450...heme synth induced and if you have a lesion in your heme synth pathway then it will now be magnified and manifest
  28. hemolytic jaundice
    • lots destroyed RBCs
    • increased bilirubin-albumin and excretion bilirubin
    • class 1
  29. cirrhosis, infectious hepatitis
    • damaged liver, affects step 3 (less bilirubin and glucuronoside)
    • increased bilirubin albumin
    • decreased bilirubin glucuronides and excretion bilirubin
    • class 2
  30. neonatal jaundice
    • immature liver (affects last step)
    • increased bilirubin albumin
    • decreased bilirubin glucoronides, excretion bilirubin
    • rx: UV to kill bilirubin
    • class 2
  31. Crigler-Najjar syndrome 1
    • complete loss UDGPT (liver, last step)
    • increased bilirubin albumin
    • decreased bilirubin-glucoronides and excretion bilirubin
    • class 2
  32. Crigler-Najjar Syndrome 2
    • UDGPT is only 10% normal (liver, last step)
    • encelopathy
    • rx: UV, heme-oxy inhib (step 1), phenobarbital (inc synth)
    • clas 2
  33. Gilbert syndrome
    • UDGPT is 50% normal (liver, last step)
    • jaundice
    • rx: phenobarbital
    • class 2
  34. obstructive jaundice
    • tumor, gall stones prevent bile to SI
    • increased bilirubin-glucoronside
    • decreased excretion bilirubin
    • class 3
  35. glycerol
    • 2 FA
    • phosphate 2/ OH
  36. sphingosine
    • 1 FA
    • phosphate w/ choline or mono/oligs
  37. pro-chiral
    L-glycerol-3-phosphate does not have an asymmetric C but once you add Pi, it becomes chiral
  38. phosphatidic acid
  39. glycerophosphoethanolamine
    • X=ethanolamine (ethyl and ammonia)
    • neutral charge
  40. glycerophosphocholine
    • X=choline (ethyl, N w/ 3 CH3)
    • neutral charge
  41. glycerophosphoserine
    • X=serine (O- and ammonia)
    • -1 charge
  42. glycerophosphoglycerol
    • X=glycerol (2 -OH)
    • -1 charge
  43. glycerophosphoinositol 4, 5 bisphosphate
    • X=myo-inositol 4,5 bisphosphate (6 membered ring 2/ 2 Pi)
    • -4 charge
  44. glycerophosphocardiolipin
    • X=phosphatidylglycerol
    • -2 charge
  45. plasmalogens
    • ether linked alkeynl chain
    • ester chain
    • X=choline
  46. platelet activating factor
    • ether linked alkyl C1
    • acetic ester linked C2
    • X=choline

    stim platelet aggregation and serotinin release, infl, allergy
  47. sphingolipids
    • sites of recog
    • neuronal PM
    • carb moeities define blood groups
    • types/Xs gangliosides changes in dev
  48. sphingoceramide
  49. spingomyelin
  50. neutral glycolipids/sphingoglucosylcerebroside
  51. sphingolactosylceramicide (globoside)
  52. spingoganglioside GM2
    X=complex oligosaccharide
  53. glycosphingolipids
  54. O blood group
  55. A blood group
  56. B blood group
  57. PLA 1
    breaks C-O bond on C1 in PL/SL degradation
  58. PLA 2
    breaks C-O bond on C2 in PL/SL degradation
  59. PLC
    breaks O-P bond off C3 in PL/SL degradation in PIP45
  60. PLD
    breaks P-O ether bond in PL/SL degradation in PIP45
  61. ganglioside degradation
    eventually yields ceramide
  62. Niemann-Pick
    • def sphingomyelinase
    • sphingomyelin accum in liver, spleen, brain
  63. Tay-Sachs
    • def hexoaminadase
    • ganglioside GM2 accum in brain, spleen
  64. Fabry's
    • def alpha galactosidase A
    • A globoside accum
  65. Sandhoff's
    • def hexosaminidase A and B
    • A globoside accum
  66. Gaucher's
    • def glucocerebrosidase
    • glucosylcerebroside accum
  67. generalized gangliosidosis
    • def beta galactosidase
    • GM1 accum
  68. eicosanoids
    • paracrines from arachadonic acid
    • PLA2 cleaves arachadonic PL at C2 ester bond
  69. arachadonic acid
    • C2 unsat at 5, 8, 11, 14
    • precursor for prostaglandin E1, thromboxane A, leukotriene A4
  70. NSAIDS
    • aspirin, naproxen, ibuprofen
    • block formation of prostaglandins, thromboxanes from arachidonic via inhib cycloxygenase
  71. cyloxygenase
    PGH2 synthase
  72. respiratory distress syndrome
    can't make enough dipalymitoyl phosphatidylcholine aka lung surfactant
  73. lysosomal degradation of prot
    • pH 5
    • non selective in nourished cells
    • selective for prot w/ KFERQ seq in starving cells
  74. ubiquitin
    • 76 AA
    • C term Gly attaches to epsilon AA on prot
    • additional ubiquitin adds at Lys 48
  75. HPV
    act E3 prot, ubiquitinates p53
  76. proteasome for ubiquitin
    • 20S
    • 28 subunits; 14 alpha, 14 beta
    • forms a sealed barrel
    • N term Thr has enz act
    • 19S cap controls access to 20S
  77. N term rule
    residue at N determ 1/2 life of prot
  78. cyclin destructive boxes
    AA mark cell for destruction
  79. pest seq
    prot w/ Pro, Glu, Ser, Thr
  80. subst spec of E3 in ubiquitin
    • N term rule
    • cyclin destructive boxes
    • pest seq
  81. aminotransferases
    contain PLP from B6
  82. PLP characteristics
    -OH that is acidic and favors deprotonation
  83. phenolate characteristics
    • tautomer of PLP
    • pyrimide ring is basic and favors protonation; has negative O
  84. schiff base intermed
    • tautomerization of PLP
    • schiff base linkage (+) stabilized by phenolate group (-)
  85. aspartate aminotransferases
    • Arg 386 orients subst by binding to alpha carboxylates
    • PLP bound to AS Lys 268 via schiff base linkage
  86. carbamoyl phosphate synthetase
    • req positive heterotropic act and N-acetylglutamate
    • high conc in mit matrix so can still func despite low aff for ammonium
  87. carbamoyl phosphate
    • 1N and 1C into urea cycle
    • removes ammonium from blood
    • Pi from ATP
  88. carbamoyl phosphate
    its phosphoanhydride bond is broken to give E to transfer of carbamoyl to orinthine
  89. citrulline
    1N, 1C from carbamoyl phosphate
  90. argininosuccinase
    completes amino transfer and keeps C skeleton from Asp
  91. arginosuccinate synthetase
    adds 2nd N from Asp
  92. arginase
    allows orinthine to cycle back
  93. urea cycle and citric acid cycle
    • connected via fumarate
    • Asp carries other AA groups into urea cycle from previous transaminations
  94. CPSI reg
    N-acetyl glutamate is allost act (signal for wanting more AA)
  95. N-acetyl glutamate
    • its conc is determ by
    • 1. acetyl CoA
    • 2. glutamate
    • 3. Arginine
  96. arginine and urea reg
    stim N-acetylglutamate synth
  97. argininosuccinase def
    • rx: low prot diet, lots Arg (bc prod of def enz)
    • as result of rx, arg excreted in urine instead of urea in order to remove ammonium
  98. CPSI or orinthine transcarbomylase def
    Gly and Glu build up

    rx: benzoate to rid Gly and phenylacetate to rid Gln
  99. ammonium toxicity
    • forms excess glutamine
    • lots glutamine and ammonium in CSF
  100. pyruvate as entry pt in AA oxid degrad
    • 3C entry
    • Ala, Ser, Cys directly
    • Thr indirectly
  101. OAA as entry pt in Aa oxid degrad
    • 4C entry
    • Asn>Asp

    uses PLP to convert alpha ketoglutarate to glutamate
  102. alpha ketoglutarate as entry pt in AA oxid degrad
    • 5C entry
    • Glu, Pro, Arg, His>glutamate>alpha ketoglutarate
  103. succinyl coenz as entry pt in AA oxid degrad
    • non polar AA entry
    • Met, Val, Ile, Thr>propionyl CoA>methylmalonyl CoA>succinyl CoA
  104. homocysteinuria
    • cystathione beta synthase def
    • no Met to succinyl CoA
  105. methyl-malonic academia
    • methylmalonyl CoA def
    • no propionyl CoA to succinyl CoA
  106. Glucogenic AA
    • AA conv to metab to can be converted to gluc
    • TCA intermed, pyruvate, all AA except others listed
  107. ketogenic AA
    • AA that give rise to ketone bodies or FFA
    • Leu, Lys
  108. glucogenic and ketogenic AA
    Ile, Phe, Tpr, Tyr
  109. Branched AA
    • Val, Ile, Leu
    • use branched aminotransferase and branched alpha ketoacid dehydratase

    primary fuels in adipose, mm, brain, kidney
  110. maple syrup disease
    • def branched alpha ketoacid dehydratase
    • rx: low Val, Ile, Leu diet
  111. monoxygenase
    1 oxygen in prod, 1 in H20
  112. phenylketonuria
    • mut in phenalanine hydroxylase
    • Phe accum
    • rx: low Phe diet
  113. tyrosinemias
    • no tyrosine aminotrans (type 2)
    • no p-hydroxyphenylpyruvate deoxygenase (type 3)
  114. alkaptonuria
    • homogentisate accum
    • dk urine
    • no homogentisate 1,2 dioxygenase
  115. Essential AA
    Met, Thr, Lys, Ile, Val, Leu, His, Phe, Tpr
  116. AA as neurotransmitters
    Glu, Gln, Asp
  117. catecholamines nt
    NE, Epi, Dopamine

    made from Tyr
  118. GABA nt
    made from glutamate
  119. histamine nt
    made from His
  120. serotonin nt
    made from Tpr
Card Set
biochemistry buzz words
MS1/Mod 4: biochemistry
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