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51 Cards in this Set
- Front
- Back
Pepsin location
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stomach
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Aminopeptidase location
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intestinal cell membrane
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Transaminase Function
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Transfer alpha-amino from AA to alpha-keto acid
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Three Transaminase Reactions
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1. Aspartate -- OAA
2. alpha-KG -- Glutamate 3. Alanine -- Pyruvate |
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Glutamate Dehydrogenase Function (3)
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1. Oxidative deamination of Glu releasing NH4+
2. Alpha-KG produced 3. NAD+ or NADP+ reduced |
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NH4 Release "Cycle"
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1. Any AA, TA, alpha-keto acid
2. a-KG takes NH3 --> Glu 3. Glu, GDH, a-KG + NH4 &NADH/NADPH |
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Deamination Special Cases
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AA with OH group can be directly deaminated, by Ser/Thr Dehydratase
1. Dehydration, form C=C 2. Hydration + Oxidation, NH4 released |
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Urea Cycle
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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 |
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Urea Cycle, which RXN in Mitochondria
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Carbamoyl-(P) + Ornitine --> Citrulline
*Citrullin then enters cytoplasm |
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Urea Cycle, source of Nitrogen (2)
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1. NH4/Carbamoyl-(P)
2. Aspartate |
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Fumarate from Urea Cycle
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Fumarate --> Malate --> OAA --> Aspartate (returns to Urea Cycle)
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AA Catabolism, 3-C group (3, 2, 1) & intermediates
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3C- Ala, Ser, Cys
Gly (Ser) & Thr (Aminoacetone) Trp (Ala) |
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AA Catabolism, 3-C Entry Point and G/K
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Pyruvate, Glucogenic
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AA Catabolism, 4-C Linear OAA Group
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Asp, Asn
Asn hydrolyzed to Asp by asparaginase |
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AA Catabolism, 4-C Linear OAA Group, Entry and G/K
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Asp + a-KG <=> OAA + Glu
OAA, Glucogenic |
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AA Catabolism, 4-C Linear Succinyl Coa Group
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Met, Val, Ile
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AA Catabolism, 4-C Linear Succinyl Coa, Entry and G/K
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Met/Val/Ile -> Propionyl CoA --> Methylmalonyl CoA --> Succinyl CoA
*MethylMalonyl CoA mutase, unusual C2 to C3 isomerization *B12 Derv, Methylcobalamine (1/2) |
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Pernicious Anemia
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Intrisic Factor dfcn --> cobalamin absorption dfcn --> can't make purine and thymine --> Anemia
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AA Catabolism, 5-C Group
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Gln, Pro, Arg, His
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AA Catabolism, 5-C Group Entry and G/K
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Glu --> a-KG
Glucogenic |
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AA Catabolism, Branched Group
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Leu, Ile, Val
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AA Catabolism, Branched Group, Entry, G/K
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TA, Decarboxylation, Dehydrogenation, Carboxylation, Hydration, Cleavage --> Acetoacetate & Acetyl CoA
Ketogenic/Both |
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Maple Syrup Urine Disease
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"Branched-chain ketoaciduria"
- Dfct. branched-chain dehydrogenase - Managed by low Val, Ile, Leu diet |
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AA Catabolism, Both Group
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Phenylalanine
Phe --> Tyr --> p-hydroxyphenylpyruvate --> Homogentisate --> Open Ring --> Mod --> Fumarate + Acetoacetate |
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AA Catabolism, Both Group, Entry
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Fumarate (Glucogenic)
Acetoacetate (Ketogenic) |
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PKU
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Failure to hydroxylate Phe -> Phenylketouria
Managed by limiting Phe intake |
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Alcaptouria
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Inherited metabolic disorder
Absence of homogentisate oxidase, can't open ring --> homogentisate accum. --> enter urine & oxidize --> black urine |
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10 Essential AA
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PVT TIM HALL
Phe, Val, Thr, Trp, Ile, Met, His, Arg, Leu, Lys |
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Biosynthesis of Serine
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From 3-phosphoglycerate, oxidation, TA, dephospho
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Biosynthesis of Glycine
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From serine + THF, two-way
THF <=> N5,N10-Methylene-THF |
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Biosynthesis of Cysteine
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1. Met + ATP -> S-AdenosylMet
2. Demethylation & hydrogenation -> Homocysteine *Methylcobalamine involvement(2/2) 3. Homocysteine + Serine -> a-Ketobutyrate + Cysteine |
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Biosynthesis of Tyrosine
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Phenylalanine +OH
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Biosynthesis of Aspartate
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OAA transamination
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Biosynthesis of Asparagine
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Aspartate + Glutamine
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Biosynthesis of Glutamate
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a-KG transamination
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Biosynthesis of Glutamine
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Glutamate + NH2
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Biosynthesis of Proline and Arginine
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From Glu semialdehyde
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Nitrogen Balanced
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Healthy, all excess excreted
Intake = Excreted |
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Positive Nitrogen Balance
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Excreted < Intake
- Growing or recoverying |
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Negative Nitrogen Balance
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Excreted > Intake
- Fasting, low protein intake --> protein broken down for essential protein synthesis, nitrogen excreted in process |
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Biomolecules from AA (5)
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NO, Creatine Phosphate, Glycocholate, Porphyrin, Neurotransmitter
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Biosynthesis of NO
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Arg + NADPH + NOS -> Citrulline + NO
*binds guanylate cyclase receptor, raise cGMP --> smooth muscle relax. eg. Viagra |
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Biosynthesis of Creatine Phosphate
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Gly -> Creatine -> Creatine Phosphate
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Biosynthesis of Glycocholate
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Glycine + Cholesterol
*Bile salt, powerful detergent |
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Biosynthesis of Porphyrin
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Glycine + Succinyl CoA
*part of heme *bilirubin and biliverdin |
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Biosynthesis of Neurotransmitter
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1. Tyrosine --> dopamine, norepinephrine
2. Tryptophan --> 5-hydroxytrypamine 3. Histidine --> histamine 4. Choline --> ACh 5. Glutamate --> GABA |
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Catabolism of Catecholamine
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1. Oxidation by MAO (monoamine oxidase)
2. Methylation by COMP (catecholamine-O-methyl transferase) |
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Simple Decarboxylation Examples (3)
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1. Histidine --> Histamine
2. Ornithine --> Putrescine 3. Lysine --> Cadaverine |
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AA Biosynthesis Regulation
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Feedback Inhibition
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AA Catabolism Cycle
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Pyruvate -> Acetyl CoA -> AcetylAcetoCoa
OAA->Citrate->α-KG->SuccinylCoA->Fumarate->OAA OAA->PEP->Glucose |
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Failure to Phe -> Tyr leads to?
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Pheylketouria
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