Metabolism

Question 1

• Sulfer AA’s? (2) Nitrogen AA’s? (2) Acidic? (2) Basic?(3)
  1. ) aKG + AA = ? (2) Enzyme? Cofactor? Key for cofactor?
  2. ) Ala + aKG = ? Enzyme?
  3. ) Glutamate + OAA = ? Enzyme? Cofactor?
  4. ) Glutatate –> ? Enzyme? Backwards uses? +? (3) -? (3)
• AA degradation occurs when?
• Tissues release? Muscle? Liver?
• Key regulated steps?
• Hydroxylation occurs with?
• Carboxylation of?
• Vit B6: Used as? Converted to? Involved in? (2)

Answer 1

• Cysteine/ methionine; Glutamine, Asparagine; Glutamic, Aspartic; Histidine, Arginine, Lysine
  1. ) Glutamate + aKA; B6/PLP; Aldehyde
  2. ) Glutamate + Pyruvate; ALT
  3. ) Aspartate + aKG; AST; B6/PLP
  4. ) aKG + NH3; Glu DH; NADPH, ADP, GDP; NAD+, ATP, GTP
• Low energy
• Glutamine, Alanine, Urea
• Glu DH, CPS1
• Pro/Lys
• Glutamic acid
• Schiff base; PLP; Niacin synthesis, Heme synthesis

Question 2

• Protein degradation: 2 cellular pathways? ATP?
• Proteases do what? Activates trypsin?
• Glucogenic means?
• Ketogenic AA’s?
  1. ) MSUD: High [ ] of? 2 steps? Defic? Leads to?
  2. ) Hyperhomo: Caused by low? (3) CV problems?
  3. ) Homocytenuria: Defect in? Treat with?
• Phe –> Tyr –> ? (2) Enzymes? Tyr involved in? (3)
  4. ) PKU defect in?
  5. ) Tyrosinemias defect where?
  6. ) Parkinsons loss of?

Answer 2

• Ubiq proteosome (ATP depend.) Lysosomal (Ind.)
• Zymogen –> enzyme; Enterpeptidase
• AA can be used for GNG
• Leucine/Lysine
  1. ) Branched AA’s; Deaminase, decarbox.; Decarbox; High keto acids
  2. ) B6,9,12; yes
  3. ) CBS; B6
• Fum + Acetoacetyl CoA; PH; Melanin, Catachol, Tissue
  4. ) PH
  5. ) After Tyrosine
  6. ) Dopamine

Question 3

• 2 Ammonia Transports in blood?
• Thyroxine: Tg –> T4 how? –> T3 how? Effect of TSH?
• Heme metabolism steps? (3)
• Sulfur AA’s? Helps with?
• Important for 3rd properties of AA?
• GSH? Product of? 4 functions?
• Met + ATP –> ? Enzyme? Used for? (3)
• Met –> SAM –> SAH –> Homo + Serine –> ? (Enzyme)
• THF come from? Used when?
• Involved in Homo –> Met? (2)
• Involved in Homo –> Cysteine?
• Tryptophan important for? (3)
• Tyrosine important for? (3)

Answer 3

• Glutamine, Alanine
• Remove Tyr Iodine; Remove another I; Incease I and T3/T4 release
• Pyrole –> Bili –> Porphoryin
• Met/ Cys; Disulfide bonds
• Cys
• Glutathione; SH buffer, Cofac, Reduce H2O2, Heme redox
• SAM (SAM synthase); Store ATP, methyl donor/transfer
• Cystathionine ( CBS)
• Folate (B9); Homo –> Met
• THF, B12
• B6
• Seratonin, Melatonin, Niacin
• Melanin, Catachol., Tissue Proteins

Question 4

• Purines? (2) Pyrimidines? (2)
• Purines: Atoms? (5) Cofactors? (4) Source of energy? (2) Source of base? Intermediate?
• Pyrimidine: Atoms? (3) Cofactors? (3) Source of energy? (1) Source of base? Intermediate?
• Sources of base?
• Purine Synthesis?
• Pyrimidine synthesis?
• Methotrexate does what?
• Ribose –> Deoxyribose enzyme? Activity site: Active by? Inhibited by? Substrate site: Reg by?
• Pur/pyr synthesis occurs where?

Answer 4

• AG; CUT
• Glu/Gly/Asp/THF/CO2; THF, CO2, ATP, AA; GTP/ATP; IMP
• Glu/Asp/CO2; ATP/PRPP/CO2; ATP; UMP
• Purine = Build base on sugar
• Pyr = Make base seperate from sugar
• Charts
• DHF –> THF blockage
• Ribonucleotide reductase; ATP; dATP; dTP/TP stuff
• Intestinal mucosal cells

Question 5

1. ) Gout: Deposition of? Often due to? Increased risk with? Can also be due to? Under parallel light?
2. ) SCID: Mutation in gene encoding what? Leads to? Which inhibits? Meaning? Effects what cells?
3. ) Lesch- Nyhan: Deficient in? Defective? PRPP? Leads to? (3) Genetics? 3 PE findings?
   - Degradation pathway: Purine N-Tide –> Remove base from? (Enzyme) –> Free Base –> ? –> Uric Acid

Answer 5

1. ) Monosodium urate crystals; undersecretion; Thiazides; overproduction of purine NTides (high degrad/uric); neg
2. ) Adenosine deaminase in purine dergrad. path; high dATP; RN reductase; low dNTPS; rapid prolif
3. ) HGPRT; purine salvage; High; High denovo purine, degrad and uric acid; XL; Mental, gout, mutilation
   - ADA, Sugar; Xanthine Oxidase

Question 6

Drugs:
dUMP –> dTMP needs what?
1.) 5FU: Pyr analog that inhibits what? Effect? Cell div?
2.) Methotrexate: Folic acid analog inhibits? Effect? Protein synthesis?
3.) 6 Mercaptoputine: Purine analog inhibits? Blocks what?
4.) Azidothymidine: Does what?
5.) ACV: Targets viral DNA polymerase and?

Answer 6

• THF
  1. ) Thymidylate synthase; low dTMP; Interferes
  2. ) DHF reductase; low dTMP; Interferes
  3. ) AMP synthesis; de novo purine synthesis
  4. ) Inhibit viral polymerase
  5. ) Reverse transcriptase