Biochem Block 3

  1. Which Sphingolipidosis produce cherry red macula?
    • G1 gangliosidosis
    • Tay-Sacchs
    • Sandoff
  2. What enzyme is deficient in GM1 gangliosidosis?
    What substrate accumulates
    • Beta galactosidase
    • GM1 ganglioside
  3. What enzyme is deficient in Tay-Sacchs?
    What substrate accumulates?
    • N-acetyl hexosaminidose
    • GM2 ganglioside
  4. What enzyme is deficient in Sandoffs?
    What substrate accumulates?
    • N-acetylhexosaminidase A and B
    • GM2 ganglioside and globoside
  5. In what sphingolipidosis are pain in lower extremities, reddish purple rash, and treatment with enzyme replacement possible?
  6. Which sphingolipidoses produces lipid laden(crumpled tissue paper) appearance?
  7. What local molecule released by active platelets promotes platelet aggregation and constriction of blood vessels in the area of injury?
    Thromboxane A2
  8. Vascular endothelial cells produce what compound that prevents platelet aggregation and vasodilates blood vessels?
  9. PGF2 is a constrictor or dilator of blood vessels?
  10. PGE2 is a vaso- constrictor or dilator?
  11. What enzyme produces Prostoglandins and Thromboxane A2?
  12. What enzyme produces Leukotrienes?
  13. What are the precursors to prostoglandins, thromboxanes and leukotrienes?
    Linoeic acid(18 carbons)----arachidonic acid( 20C)
  14. Conversion of Arachidonic acid to prostoglandins is done by what enzyme?
    PG endoperoxide synthase (PGES)
  15. What are the two isoenzymes of PG endoperoxide Synthase?
    COX-1 and COX-2
  16. What is the difference between COX 1 and COX 2?
    • COX 1 is expressed constitutively abundantly and is involved in maintenance of gastric mucosa, renal homeostasis and platelet aggregation
    • COX 2 is inducible involved in in heat pain and inflammation
  17. What enzyme converts membrane lipid( phosphotidylcholine) to arachidonic acid?
    Phospholipase A
  18. What prevents the action of phospholipase A2 and COX-2?
  19. What effect does aspirin have on platelet aggregation and cox 1?
    irreversibly binds COX-1 and reduces platelet aggregation
  20. What drug inhibits only COX-2?
  21. What acts as a neutrophil chemotactic agent?
  22. Leukotrienes LTC4, D4, E4 function to do what?
    • increase vascular permeability
    • bronchoconstriction
    • vasocontriction
    • contraction of smooth muscle
  23. Endoperoxides give rise to what 3 distinct group of signalling molecules?
    • Prostocyclin--inhibit platel gatherin, vasodilation
    • Prostoglandin---pain, uterine tone, temperature
    • Thromboxanes- promote platelet aggregation, vasoconstriction
  24. Heparin inhibits synthesis of what factors?
    thrombin, 9,10,11,12
  25. Warfarin inhibits the synthesis of what clotting factors?
    Protein C, S, Vitamin K derived clotting factor--2,7,9,10
  26. What is the rate limiting step for FA synthesis?
    Acetyl coa Carboxylase
  27. What is the rate determining enzyme for FA oxidation?
    Carnitine acyl transferase I
  28. Rate limiting step for HMP shunt?
    G6PD--glucose 6 phosphate dehydrogenase
  29. Rate limiting step for TCA?
    isocitrate dehydrogenase
  30. Rate limiting step for glycolysis?
  31. Rate limiting step for cholesterol synthesis?
    HMG coA reductase
  32. What is the rate limiting step for glycogen lysis?
    glycogen phosphorylase
  33. Rate limiting step for gluconeogenesis?
    Fructose 1,6 bisphosphatase
  34. Rate limiting step for glycogen synthesis?
    glycogen synthase
  35. Why doesnt glucokinase have an allosteric inhibitor?
    • because it has a high Km/low affinity for glucose
    • acts as a glucose buffer in the blood
  36. Which glucose transporter is insulin sensitive?
  37. Which GLUT is needed for fructose transport?
  38. What is the structure of Acetyl coA Carboxylase?
  39. Desaturation of fatty acids occurs where?
  40. What 2 components are used for desaturation of FA?
    • oxygen
    • NADH
  41. On the glycerol back bone what is the nature of FA on C1
    • C1 saturated FA
    • C2 Unsaturated FA
    • C3 sat or unsaturated
  42. What is the source of NADPH for FA synthesis?
    • 2 NADPH from HMP shunt
    • 1 NADPH from malate to pyruvate
  43. Fatty acid synthase produces what kind of FA?
  44. What inhibits carnitine acyltransferase I?
    Malonyl coA
  45. How does liver synthesize Glycerol 3 Phosphate?
    Using glycerol Kinase
  46. How does Adipose tissue synthesize glycerol 3 phosphate
    Glucose is broken down to dihyroxyacetone phosphate which is reduced to glycerol 3 phosphate
  47. What is the activator of lipoprotein lipase?
    apoprotein CII
  48. Why cant adipose tissue synthesize TAG when insulin is slow?
    insulin is needed for glucose uptake which is then converted to Glycerol 3 phosphate
  49. What are the two enzymes that produces NADPH?
    • Glucose 6 phosphate dehydrogenase
    • Malic enzyme
  50. Conversion of malate to pyruvate is done by what enzyme?
    Whats produced?
    • Malic enzyme
    • NADPH is produced
  51. What enzyme converts pyruvate to OAA?
    pyruvate carboxylase
  52. Addition of fatty acyl coA to glycerol 3 phosphate produces what?
    Phosphatidic acid
  53. Phosphatidic acid is converted to diacylglycerol by what enzyme?
  54. What enzyme removes FA's from C1 and C3 of TAG's?
    Hormone sensitive lipase (HSP)
  55. What enzymes phosphorylate and thus induce the action of hormone sensitive lipase?
    Glucagon, Epinephrine, Cortisol??, Thyroxine?, GH??
  56. Glycerol released from TAG's cannot be used because?
    Adipose tissue lacks glycerol kinase so it is instead transported to the liver
  57. Upon hydrolysis from TAG's, how are FA activated?
    By conversion to fatty acyl coA
  58. What is the rate limiting enzyme of FA beta oxidation?
    Carnitine acyltransferase
  59. Fatty acyl coA enzyme that activates FA? is located where?
    Outer mitochondrial membrane
  60. Carnitine acyltransferase converts fatty acylcoA to what product?
    Fatty acyl carnitine
  61. Carnitine acyltransferase I (CPT I) is located where?
    in the outer mitochondrial membrane
  62. What happens to fattyacyl carnintine that is formed in the intermembranous space?
    It is translocated into the mitochondrial matrix
  63. What enzyme converts fattyacylcarnitine to fatty acyl coA in the mitochondrial matrix?
    Carnitine acyltransferase II
  64. What happens to the carnitine that is released in the mitochondrial matrix from the enzymatic action of Carnitine acyltransferase?
    it is translocated back to the intermembraneous space
  65. What are the sources of carnitine?
    • Diet
    • Lysine
    • methionine
  66. What chain length fatty acids need carnitine for transport into the mitochondrial matrix?
  67. CAT I deficiency primarily affects what organ?
  68. CAT II deficiency primarily affects what organs?
    skeletal muscle and cardiac muscle
  69. Medium chain acyl coA dyhydrogenase (MCAD) deficiency is a deficiency of what enzyme?
    acyl coA dehydrogenase
  70. What kind of FA's provide a glucogenic intermediate?
    Odd chain Fatty acids
  71. Why does only Odd chain fatty acids provide a glucogenic intermediate?
    Because after beta oxidation, a 3 carbon molecule(propionyl coA) remains which is converted to succinyl coA
  72. Methylmalonyl coA Mutase is involved in the conversion of priopionyl CoA to succinyl coA. What is the necessary coenzyme?
    Vitamin B12
  73. Vitamin B12 is needed for what processes?
    myelination, RBC maturation, oxidation of Odd length chain fatty acids
  74. What are the products of one round of beta oxidation on a FA?
    • 1 FADH2
    • 1NADH
    • acetyl coA
  75. Methylmalonic acidemia and aciduria can be caused by what factors, what are they?
    • Vit B12 deficiency
    • Methylmalonyl coA mutase
  76. Which one is glucogenic, propionyl coA or acetyl coA?
    Propionyl coA
  77. Oxidation of unsaturated FA may require what high energy molecule?
  78. Failure to target VLCFA to peroxisome or peroxisome insufficiency results in what syndrome?
    Zellweger syndrome
  79. Failure to transport VLCFA to Peroxisome results in what disorder?
  80. What high energy molecule is used in beta oxidation in peroxisomes which produces the hydrogen peroxide radical?
  81. Refsum's disease results from deficiency of what enzyme?
    what accumulates?
    • alpha hydroxylase
    • phytanic acid
  82. What are the 3 ketone bodies?
    • acetoacetate
    • 3 hydroxybutarate
    • acetone
  83. Ketone body production occurs where?
    in the liver
  84. Under what condition does ketone body formation occurs?
    • high TAG in the blood
    • Starvation
  85. Why are ketone bodies produced?
    • oxaloacetate is shunted to gluconeogenesis
    • acetyl coA cant form citrate with OAA
    • acetyl coA is diverted to form ketone bodies
  86. Ketone bodies are made from what?
    HMG coA
  87. How are HMG coA made?
    • acetyl coA+ acetyl coA= acetoacetyl coA
    • acetoacetyl coA + acetyl coA= HMG coA
  88. Which ketone body is not metabolized?
  89. What gives rise to acetoacetate?
  90. What is hypoketotic hypoglycemia?
    low levels of ketone bodies and hypoglycemia
  91. Which ketone body is readily usable?
  92. Excretion of ketone bodies in urine causes what condition?
  93. What activates LCAT?
    apoprotein A1
  94. Ammonia is converted to what?
  95. In what situations is positive nitrogen balance?
    • children
    • pregnant women
  96. When is negative nitrogen balance seen?
    • trauma
    • illness
    • elderly
  97. What types of protein are stable?
    • structural proteins (e.g)--Hb, collagen
    • unstable---regulatory enzymes
  98. HB 27 marker on MHC predisposes individuals to what condition?
    Ankylosing spondylitis or other inflammatory conditions
  99. Cholesystokinin and secretin are secreted in response to what in the stomach?
  100. Zymogens are released to prevent what?
  101. Why is digestion not normal in cystic fibrosis?
    hepatopancreatic duct is clogged up with thick secretions so no enzymes reach the intestine--poor digestion
  102. Kwashiorkor
    • protein deficiency
    • sufficient calories
  103. Marasmus
    • protein deficiency
    • calorie deficiency
    • severe muscle wasting
  104. What converts pepsinogen to pepsin?
    H + in the stomach
  105. What converts trypsinogen to trypsin?
    enteropeptidase (enterokinase)
  106. What are serine proteases?
    protein digesting enzymes
  107. Trypsin cleaves what a.a?
    • Basic a.a
    • lysine
    • arginine
  108. Chemotrypsin cleaves what?
    hydrophobic or acidic AA
  109. Elastase cleaves what?
    • A.A with small side chains
    • ala, gly. ser
  110. What are the 3 exopeptidases
    • Carboxypeptidase A, B
    • Aminopeptidases
  111. How is the transport of A.A differ across the luminal and serosal surface?
    • lumen-- Na+ cotransport ATP used
    • serosa-- facilitated diffusion
  112. Deficiency of neutral amino acids transport causes?
  113. Cystinuria or dibasic aciduria is caused by a deficiency of what a.a?
    cystine, ornithine, lysine, Arginine
  114. Dicarboxylic aciduria is caused by a deficiency of what a.a?
    glutamate, aspartate
  115. Joseph's syndrome or glycinuria is caused by?
    gly, pro, hyprolinen
  116. Hartnup has what conditions?
    • Dementia
    • Diarhea,
    • Dermatitis
  117. Cystinuria occurs bc of faulty transport of a.a where?
    epithelia brush border of sm. intestine and renal tubules
  118. Excess cysteine in blood can lead to ?
    Kidney stones
  119. In a transamination, what new a.a is formed?
  120. During starvation, glutamate is converted to what?
    alpha keto glutarate
  121. Peroxisomes have what amino acid oxidase?
    • L-amino acid oxidase
    • source of hydrogen peroxide
  122. D-amino acid oxidase is used to degrade proteins from what source?
  123. During Transamination, all nitrogen is tunneled into what a.a?
  124. Alpha amino group is moved from what to what?
    donor amino acid to an acceptor alpha keto acid
  125. What vitamin is needed for transamination?
    pyridoxal phosphate B6
  126. Aminotransferases exist for all amino acids except what?
    • threonine
    • lysine
  127. Apartate amino transferase and alanine aminotransferase are clinically important, why?
    Indicates hepatic disease
  128. What enzymes would be present in hepatic cell injury?
    • AST
    • ALT
  129. Gamma glutymal transferase
    alcoholic liver cirhosis
  130. Cholestatic lesion is caused by?
    bile duct obstruction
  131. Alkaline phosphatase is a marker for what two conditions?
    • cholestatic lesions
    • bone disease
  132. Non-oxidative deamination is seen in what two a.a deamination?
    • serine
    • threonine
  133. Glutamate dehydrogenase utilizes what two high energy carriers?
    • NAD+
    • NADP+
  134. Glutamate dehydrogenase converts glutamtate to what ?
    alpha ketoglutarate and ammonium ion
  135. Xanthine oxidase produces what?
    hydrogen peroxide
  136. Urea cycle takes place where?
  137. The nitrogen atoms in urea come from what 2 sources?
    • Aspartate
    • free NH4+
  138. The Carbon in urea comes from ?
  139. Carbamoyl Phosphate I is involved in what cycle?
  140. What is the rate limiting step of the urea cycle?
    Carbamoyl Phosphate synthetase I
  141. Formation of ornithine takes place where in the cell?
  142. After citrulline is formed in the mitochondria what happens to it?
    it enters the cytoplasm
  143. What is the allosteric activator of Carbamoyl phosphate synthetase I?
  144. How is nitrogen amount in the blood measured?
    amount of nitrogen divided by the blood's urea (e.g.......14/60)
  145. What is used to screen for kidney function?
    BUN and creatine
  146. Wilson's disease?
    • Copper accumulation in the liver
    • causes chorea form movements
    • caudate deposition of copper
  147. Kwashiokor condition results from protein deficient MEAL?
    What does the MEAL stand for
    • Malnutrition
    • Edema
    • Anemia
    • Liver change (fatty) decreases lipoprotein synthesis
  148. What converts pepsinogen to pepsin?
  149. What converts Trypsinogen to trypsin?
    enteropeptidase (enterokinase)
  150. Deficiency of absorption of neutral a.a like tryptophan results in what condition?
    • Hartnup (Pellagra)
    • Dementia
    • Diarhea
    • Dermatitis
  151. Which a.a are not absorbed in the PCT of the kidney and as a result cause Cystynuria?
    • cysteine
    • Ornithine
    • Lysine
    • Arginine
  152. What a.a is present in excess in the kidney in Cystynuria?
  153. Absorption deficiency of cysteine in Cystynuria can cause what?
    Kidney stones
  154. Defective neutral a.a transporter on kidney tubules and intestinal cells causes what condition?
    Hartnup disease
  155. Which a.a undergo non-oxidative deamination?
    • serine
    • threonine
  156. Upon deamination, aspartate becomes what?
  157. Aminotransferases exist for all amino acids except 2, what are they?
    • Serine
    • Threonine
  158. In a transamination reaction,what does
    aspartate become?
    alpha ketoglutarate become?
    • aspartate---> oxaloacetate
    • alpha ketoglutarate----> glutamate
  159. Transaminases use what cofactor?
    pyridoxal phosphate (Vitamin B6)
  160. In a transamination rxn, what does
    alanine become
    alpha ketoglutarate
    • alanine--> pyruvate
    • alpha ketoglutarate---> glutamate
  161. What a.a give rise to alpha keto glutarate?
    • Histidine
    • Glutamate
    • Arginine
    • Proline
  162. Urinary levels of FIGLu would be high bc of a deficiency of what?
  163. What a.a give rise to pyruvate?
    • Glycine
    • Alanine
    • Serine
    • Cysteine
    • Threonine
  164. Phenylalanine hydroxylase uses what coenzyme?
  165. Sources of succinyl coa
    • Threonine
    • Isoleucine and valine
    • Methionine
  166. What accounts for the high mitochondrial mutation rate?
    No proof reading capabilities
  167. Any gene located in nucleus produces what pattern?
    Mendelian inheritance
  168. Homoplasmy
    Identical mitochondria population
  169. An example for reduced penetrance
    Split hand deformity
  170. Example of variable expression
  171. mitochondrial mutations primarily produce what clinical disease?
    neuromuscular diseases
  172. Pearson syndrome affects what type of tissues?
    tissue that depend on oxidative phosphorylation
  173. What two coenzymes are needed for SAM regeneration?
    • cobalamin
    • folate
  174. What is the most soluble porphyrin?
    • Uroporphoryin III
    • coproporphyrin III
  175. Which porphyrin is not important for synthesis of heme?
  176. Porphyrin I is elevated in what condition?
  177. Heme containing proteins is called what?
  178. Heme proteins are also present where?
    • myoglobin
    • cytochrome
    • Catalase
    • Tryptophan Pyrrolase
  179. All porphyrins have a characteristic absorption spectrum in what?
    visible and ultraviolet light
  180. 400-700 nm is what light?
    visible light
  181. What is cancer phototherapy?
    • tumor take up porphyrin
    • tissue is irradiated with UV light bc porphyrin absorb UV
  182. Biosynthesis of Heme?
    Liver and bone marrow
  183. Heme synthesis occurs where in the cell?
    • mitochondria
    • cytosol
  184. What are 3 processes that take place in mitochondria and cytosol?
    • gluconeogenesis
    • urea cycle
    • heme synthesis
  185. What are the precursors of heme?
    • Glycine
    • Succinyl coA
  186. How many molecules of glycine and succinyl coA are needed for 1 molecule of heme?
  187. What TCA cycle intermediate is used for heme synthesis?
    Succinyl coA
  188. how many molecules of acetyl coa are needed to form cholesterol?
  189. What is the rate determining step of heme synthesis?
    ALA synthase
  190. What coenzyme is needed for ala synthase
  191. 2 molecules of alpha alanine combine to form what?
  192. What enzyme forms porphobilinogen?
    ALA dehydratase
  193. 1 porphobilinogen + 3 porphobilinogens coupling is catalyzed by what enzyme?
    • hydroxymethylbilane synthase
    • uroporphyrinogen I synthase
    • Porphobilinogen deaminase
  194. What converts hydroxymethylbilane to uroporphyrinogen II?
    Uroporphyrinogen II synthase
  195. Uroporphyrin is made from what?
  196. What inhibits ALA synthase?
  197. ALAS1 ia present where?
    ALAS2 is present where?
    • Liver
    • Bone marrow
  198. At what molecular level does heme control the ALAS1 enzyme?
    • transcription
    • translation
  199. What is hemin?
    oxidized (ferric) form of heme
  200. Hemin is found where?
    methemoglobin---> chocolate cyanosis
  201. What sugar inhibts heme synthesis?
  202. Drugs have what effect on ALA synthase?
    they upregulate ALA synthase
  203. Why would drugs upregulate ALA synthase?
    cytochrome 450 uses heme and is involved in drug metabolism
  204. Lead inhibits what 2 enzymes?
    • ALA dehydratase
    • Ferrochelatase
  205. Lead poisoning causes what?
  206. Porphyrias are caused by?
    low heme presence
  207. All but one porphyrias are inherited in what manner?
    Autosomal Dominant
  208. Porphyrias could be of 2 kinds?
    What are they?
    • Erythropoietic
    • Hepatic
  209. Acute type of porhyria is characterized by what?
    • abominal pain
    • neuropsychiatric symptoms
  210. What are the symptoms of cutaneous type of porphyria?
    • photosensitivity
    • cutaneous lesions
  211. Acute intermittent porphyria
    • Uroporphobilinogen I synthase
    • Porphobilinogen deaminase
  212. Congenital erythropoietic Porphyria is caused by what?
    uroporphobilinogen III synthase
  213. Uroporphyrin I is found in what type of porphyria?
    Congenital erythropoietic porphyria
  214. Protophyria caused by a deficiency of what enzyme?
  215. What diminshes photosensitivity?
    beta carotene
  216. X linked sideroblastic anemia is caused by what?
    Deficiency of ALA synthase 2
  217. What accumulates in Refsum disease?
    phytanic acid---branched chain FA
  218. What is the defect in Wilson disease?
    copper transport
  219. Kayser-Fleisher ring is seen in what condition?
  220. Treatment for Wilsons desease
  221. Cause for Menke's Syndrome
    Efflux of copper
  222. What 2 acids undergo non oxidative deamination?
    • threonine
    • serine
  223. What two a.a do not have transaminases?
    • threonine
    • lysine
  224. Oxidative deamination is catalyzed by what enzyme?
    glutamate dehydrogenase
  225. D amino acid oxidase uses what high energy coenzyme?
  226. What are the 2 ways of transporting ammonia?
    • glutamine
    • alanine from pyruvate
  227. Amino acid oxidases rxn take place where in the cell?
  228. Glutamate dehydrogenase (oxidative deamination) takes place where in the cell?
  229. Formation of alanine from pyruvate is done by what enzyme?
    Alanine transferase
  230. The 2 nitrogens and 1 carbon in urea come from where?
    • N-aspartate via glutamate
    • N--free ammonium ion
    • C--carbon dioxide
  231. How many ATP's are used in Urea cycle?
  232. Argininosuccimic acidemia is caused by a deficiency of what enzyme?
    Arginosuccinate lyase
  233. What substrate accumulates in Fructose intolerance?
  234. Fructose intolerance is caused by deficiency of what enzyme?
    aldolase B
  235. Essential fructosuria is caused by a deficiency of what enzyme?
  236. Classic galactosemia is caused by what?
    galactose 1 phosphate uridyltransferase
  237. Glucose 6 phosphatase is found where in the cytosol?
    What organ?
    ER in the liver
  238. 2 Uncoupling agents?
    • Aspirin
    • 2,4 DNP
  239. Accumulation of fructose 1 phosphate inhibits what pathway?
    • Gluconeogenesis
    • Glycogenlysis
  240. High levels of galactose and/or fructose result in what condition?
    • change to galacticol and fructisol
    • which are osmotically active
  241. High levels of ammonium ion have what effect on the TCA cycle?
    it depletes alpha ketoglutarate inhibiting TCA cycle
  242. What is the only phospholipid with antigenic properties?
  243. Plasmalogens are found where?
    nerve tissue
  244. Cardiolipin is found where?
    Inner mitochondrial membrane
  245. Sphingomyelin has what backbone?
    amino alcohol
  246. Which glycosphingolipids are common in membranes?
  247. Paroxysmal Nocturnal Hemoglobinuria is caused by?
    PIGA gene which is needed for synthesis of GIP anchors
  248. What is a symptom of Paroxysmal Nocturnal Hemoglobinuria?
    Hemolytic Anemia
  249. What phospholipid is deficient in Paroxysmal Nocturnal Hemoglobinuria?
  250. What are the 3 types of Glycosphingolipids?
    • Globosides- N acetyl galactosamine
    • Cerebroside
    • Ganglioside--NANA
  251. What degrades Phospholipids?
  252. Glycerophospholipids are synthesized from where?
    • Glycerol 3 phosphate
    • phosphatidic acid
  253. Phosphatidylinositol is broken down into what two components?
    IP3 and DAG
  254. Digestion of lipids begins where?
    Using what enzyme?
    • stomach
    • lingual lipase
  255. What are two enzymes that degrade short to medium chain fatty acids?
    Where do they act?
    • Gastric lipase
    • Lingual Lipase

  256. What is the structure of bile salts?
    sterol ring with taurine or glycine attached to it
  257. Increasing chain unsaturation does what to melting point?
    it decreases it
  258. Which FA is needed for prostoglandin synthesis if arachidonic acid is not present?
  259. The R1, R2, R3 is what in a TAG?
    • R1-saturated
    • R2-unsaturated
    • R3-either
  260. Human milk is rich in what TAGs?
    Short and medium length fatty acids
  261. Pancreatic lipase converts TAG's to what?
    • 2 monoacylglycerol
    • 2 free fatty acids
  262. What activates Lipase?
  263. What does orlistat do?
    inhibits pancreatic lipase secretion
  264. C2 FA of phosphotidylcholine is removed by what enzyme?
    • C2---phospholipase A2
    • C1-Lysophospholipase
  265. What hormone stimulates the contraction of gall bladder?
    Release of pancreatic enzyme?
    Inhibits gastric emptying?
  266. Which FA do not need miscelles for transport across SI Wall?
    short and medium chain
  267. Where in the enterocytes are TAG resynthesizes?
  268. How are TAG resynthesized?
    FA are activated by thiokinase and added to 2-monoglycerol
  269. After cholesterol and FA are resynthesized in the ER what happens to them?
    they are packed into a chylomicron with apo B48
  270. Type I hypercholesterolemia is caused by what?
    deficiency of Lipoprotein lipase or its activator apo protein C II
  271. What leads to Familial Hyperlipoproteinemia type III?
    deficiency of uptake of chylomicrons remnants
  272. What a.a is used to form N-glycosidic bonds in glycoproteins?
  273. FIGLu excretion in urine is a symptom of deficiency of what coenzyme?
  274. What acts as a high energy reserve molecule in the muscle?
    Creatine phosphate
  275. Non ketotic HyperGlycynemia is caused by what?
    • deficient glycine degradation
    • high glycine in blood
  276. Deficiency of what enzyme causes non ketotic Hyperglycenemia?
    Glycine cleavage complex
  277. Primary Hyperoxaluria caused by?
    increased conversion of glycine to oxalate
  278. Glycinuria is caused by?
    defective absorption of glycine in kidney
  279. Biliverdin thats bound to albumin dissociates from albumin in the liver and binds to what?
  280. What is needed for the conversion of heme to biliverdin?
    NADPH, O2 and Fe
  281. Biliverdin formation takes place where in the cell?
  282. Salicylates and sulfanamides have what effect on the albumin-bilirubin complex?
    it dissociates it, freezing bilirubin which can cross BBB and cause kernicterus
  283. What is the rate limiting step in bilirubin excretion?
  284. What does MOAT do?
    Transports bilirubin into canaliculi
  285. What drug increases the synthesis of UDP-Glucoronyl transferase?
  286. What is Gilberts syndrome?
    • Partially deficient UDP glucuronyl transferase enzyme
    • deficient uptake of bilirubin
    • unconjugated bilirubin
  287. Crigler-najjar syndrome?
    • Deficient UDP glucuronyl transferase
    • unconjugated bilirubin
  288. Dubin Johnson and Rotor syndrome are what?
    deficient excretion of conjugated bile
  289. Alkaline phosphatase is a marker of what two physiological sites?
    • bones
    • liver
  290. What is the direct reacting bilirubin?
    conjugated one
  291. What is the indirect reacting Bilirubin?
  292. Which drug has combined effects of pharmokinetics and dynamics?
Card Set
Biochem Block 3
Biochem block 3