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51 Cards in this Set

  • Front
  • Back
Pepsin location
stomach
Aminopeptidase location
intestinal cell membrane
Transaminase Function
Transfer alpha-amino from AA to alpha-keto acid
Three Transaminase Reactions
1. Aspartate -- OAA
2. alpha-KG -- Glutamate
3. Alanine -- Pyruvate
Glutamate Dehydrogenase Function (3)
1. Oxidative deamination of Glu releasing NH4+
2. Alpha-KG produced
3. NAD+ or NADP+ reduced
NH4 Release "Cycle"
1. Any AA, TA, alpha-keto acid
2. a-KG takes NH3 --> Glu
3. Glu, GDH, a-KG + NH4 &NADH/NADPH
Deamination Special Cases
AA with OH group can be directly deaminated, by Ser/Thr Dehydratase
1. Dehydration, form C=C
2. Hydration + Oxidation, NH4 released
Urea Cycle
0. NH4 from TA & GDH
1. NH4+CO2, Synthetase, Carbamoyl-(P)
2. Ornitine + Carbamoyl-(P) --> Citrulline
3. Citrulline + Asp --> Argininosuccinate
4. Arginosuccinate --> Arg + Fumarate
5. Arg + H2O --> Ornitione + Urea
Urea Cycle, which RXN in Mitochondria
Carbamoyl-(P) + Ornitine --> Citrulline
*Citrullin then enters cytoplasm
Urea Cycle, source of Nitrogen (2)
1. NH4/Carbamoyl-(P)
2. Aspartate
Fumarate from Urea Cycle
Fumarate --> Malate --> OAA --> Aspartate (returns to Urea Cycle)
AA Catabolism, 3-C group (3, 2, 1) & intermediates
3C- Ala, Ser, Cys
Gly (Ser) & Thr (Aminoacetone)
Trp (Ala)
AA Catabolism, 3-C Entry Point and G/K
Pyruvate, Glucogenic
AA Catabolism, 4-C Linear OAA Group
Asp, Asn
Asn hydrolyzed to Asp by asparaginase
AA Catabolism, 4-C Linear OAA Group, Entry and G/K
Asp + a-KG <=> OAA + Glu
OAA, Glucogenic
AA Catabolism, 4-C Linear Succinyl Coa Group
Met, Val, Ile
AA Catabolism, 4-C Linear Succinyl Coa, Entry and G/K
Met/Val/Ile -> Propionyl CoA --> Methylmalonyl CoA --> Succinyl CoA
*MethylMalonyl CoA mutase, unusual C2 to C3 isomerization
*B12 Derv, Methylcobalamine (1/2)
Pernicious Anemia
Intrisic Factor dfcn --> cobalamin absorption dfcn --> can't make purine and thymine --> Anemia
AA Catabolism, 5-C Group
Gln, Pro, Arg, His
AA Catabolism, 5-C Group Entry and G/K
Glu --> a-KG
Glucogenic
AA Catabolism, Branched Group
Leu, Ile, Val
AA Catabolism, Branched Group, Entry, G/K
TA, Decarboxylation, Dehydrogenation, Carboxylation, Hydration, Cleavage --> Acetoacetate & Acetyl CoA
Ketogenic/Both
Maple Syrup Urine Disease
"Branched-chain ketoaciduria"
- Dfct. branched-chain dehydrogenase
- Managed by low Val, Ile, Leu diet
AA Catabolism, Both Group
Phenylalanine
Phe --> Tyr --> p-hydroxyphenylpyruvate --> Homogentisate --> Open Ring --> Mod --> Fumarate + Acetoacetate
AA Catabolism, Both Group, Entry
Fumarate (Glucogenic)
Acetoacetate (Ketogenic)
PKU
Failure to hydroxylate Phe -> Phenylketouria

Managed by limiting Phe intake
Alcaptouria
Inherited metabolic disorder
Absence of homogentisate oxidase, can't open ring --> homogentisate accum. --> enter urine & oxidize --> black urine
10 Essential AA
PVT TIM HALL
Phe, Val, Thr,
Trp, Ile, Met,
His, Arg, Leu, Lys
Biosynthesis of Serine
From 3-phosphoglycerate, oxidation, TA, dephospho
Biosynthesis of Glycine
From serine + THF, two-way

THF <=> N5,N10-Methylene-THF
Biosynthesis of Cysteine
1. Met + ATP -> S-AdenosylMet
2. Demethylation & hydrogenation -> Homocysteine *Methylcobalamine involvement(2/2)
3. Homocysteine + Serine -> a-Ketobutyrate + Cysteine
Biosynthesis of Tyrosine
Phenylalanine +OH
Biosynthesis of Aspartate
OAA transamination
Biosynthesis of Asparagine
Aspartate + Glutamine
Biosynthesis of Glutamate
a-KG transamination
Biosynthesis of Glutamine
Glutamate + NH2
Biosynthesis of Proline and Arginine
From Glu semialdehyde
Nitrogen Balanced
Healthy, all excess excreted
Intake = Excreted
Positive Nitrogen Balance
Excreted < Intake
- Growing or recoverying
Negative Nitrogen Balance
Excreted > Intake
- Fasting, low protein intake --> protein broken down for essential protein synthesis, nitrogen excreted in process
Biomolecules from AA (5)
NO, Creatine Phosphate, Glycocholate, Porphyrin, Neurotransmitter
Biosynthesis of NO
Arg + NADPH + NOS -> Citrulline + NO
*binds guanylate cyclase receptor, raise cGMP --> smooth muscle relax. eg. Viagra
Biosynthesis of Creatine Phosphate
Gly -> Creatine -> Creatine Phosphate
Biosynthesis of Glycocholate
Glycine + Cholesterol

*Bile salt, powerful detergent
Biosynthesis of Porphyrin
Glycine + Succinyl CoA

*part of heme
*bilirubin and biliverdin
Biosynthesis of Neurotransmitter
1. Tyrosine --> dopamine, norepinephrine
2. Tryptophan --> 5-hydroxytrypamine
3. Histidine --> histamine
4. Choline --> ACh
5. Glutamate --> GABA
Catabolism of Catecholamine
1. Oxidation by MAO (monoamine oxidase)
2. Methylation by COMP (catecholamine-O-methyl transferase)
Simple Decarboxylation Examples (3)
1. Histidine --> Histamine
2. Ornithine --> Putrescine
3. Lysine --> Cadaverine
AA Biosynthesis Regulation
Feedback Inhibition
AA Catabolism Cycle
Pyruvate -> Acetyl CoA -> AcetylAcetoCoa
OAA->Citrate->α-KG->SuccinylCoA->Fumarate->OAA
OAA->PEP->Glucose
Failure to Phe -> Tyr leads to?
Pheylketouria