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49 Cards in this Set
- Front
- Back
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What does it mean if an AA is Glucogenic?
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Can be used to generated Pyruvate or another Intermediate of the TCA Cycle
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What does it mean if an AA is Ketogenic?
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Can be used to yield Acetoacetate, Acetyl CoA, or Acetoacetyl CoA
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What is an Essential AA? What is Nonessential AA?
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-Cannot be Synthesized
-Can be Synthesized |
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What does the Acronym "Mother Theresa's Vagina Is Pretty Tight Loving Lord"?
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Essential Amino Acids
-Methionine, Threonine, Valine (Glucogenic) -Isoleucine, Phenylalanine, Tryptophan (Glucogenic and Ketogenic) -Leucine and Lysine (Ketogenic) |
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Which AAs are Essential?
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Mother Theresa's Vagina Is Pretty Tight Loving Lord
Essential Amino Acids -Methionine, Threonine, Valine (Glucogenic) -Isoleucine, Phenylalanine, Tryptophan (Glucogenic and Ketogenic) -Leucine and Lysine (Ketogenic) |
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Which AAs from Oxaloacetate? What is the Pathway?
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-Asparagine and Aspartate
-Asparagine > Aspartate > Oxaloacetate |
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Why isn't Aspartate an essential AA?
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It is formed as an Intermediate in the Synthesis of Oxaloacetate from Asparagine
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What Catalyzes the Formation of Oxaloacetate from Aspartate? What is the functional significance of Aspartate if it doesn't participate in this reaction?
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-AST - Aspartate Aminotransferase
-Transports Nitrogen to Urea Cycle and Donates 2nd N |
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The Formation of Oxaloacetate from Aspartate is Catalyzed AST. What else must happen in this reaction?
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An Amine must be transferred to Alpha-Ketogluturate to form Glutamate
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What happens in the Acute Lymphoblastic Leukemia (ALL)?
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-Patients cannot Synthesize Asparagine
-Asparaginase converts Asparagine to Aspartate in the formation of Oxaloacetate -Need Asparagine for Cells that are growing |
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How is Acute Lymphoblastic Leukemia (ALL) treated? Why?
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-Administration of Asparaginase
-Enzyme is Deficient -Need this Enzyme to convert Asparagine to Aspartate in the formation of Oxaloacetate |
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What is FIGlu? How does it form? What is the overall pathway?
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-Intermediate in the Conversion of Histidine to Alpha-Ketogluturate
-Histidine > Urocanic Acid > FIGlu > Glutamate > Alpha-Ketogluturate |
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What catalyzes the conversion of FIGlu to Glutamate?
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THF
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What does it usually mean if FIGlu is accumulating? Why?
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-THF Deficiency
-THF if required to convert FIGlu to Glutamate |
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Which AAs form Fumurate? What is the pathway?
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-Phenylalanine and Tyrosine
-Phenylalanine > Tyrosine > Fumurate + Acetoacetate |
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Which enzyme facilitates the conversion of Phenylalanine to Tyrosine? What mediates this reaction?
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-Phenylalanine Hydroxylase
-Tetrahydrobiopterin (Vitamin) and Oxygen |
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Tetrahydrobiopterin (Vitamin) and Oxygen are used to mediated which reaction? Which enzyme is used in this reaction?
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-Phenylalanine to Tyrosine
-Phenylalanine Hydroxylase |
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What does Catabolism of Methionine result in?
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Succinyl CoA
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Which AAs form Succinyl CoA?
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-Valine, Isoleucine (Branched)
-Threonine -Methionine |
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Which Branched Chain AAs form Succinyl CoA? Which ones don't and what do they form?
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-Valine and Isoleucine
-Leucine |
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What is the Major Methyl Group Donor in the Body? Which AA is this formed from?
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-SAM - S-Adenosylmethionine
-Methionine |
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What types of reactions does SAM (S-Adenosylmethionine) participate in? What does it do in these reactions?
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-Methylation of DNA and Protein
-Donates Methyl Groups |
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What is L-Homocysteine associated with? How does it result?
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-Vascular Disease (not sure why)
-From SAM after Methyl Group is donated (2 steps after) -Originally from Methionine |
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What are the 2 Fates of Homocysteine?
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-Resynthesis of Methionine
-Synthesis of Cysteine |
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How is Methionine resynthesized from Homocysteine? When is this reaction used? What its important Mediators?
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-Homocysteine is Remethylated to give Methionine
-Used when Methionine Levels are Low -Folic Acid and Vitamin B12 |
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What are the Important Mediators of the Resynthesis of Methionine from Homocysteine?
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Folic Acid and Vitamin B12
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What role does Folic Acid play in the Resynthesis of Methionine from Homocysteine?
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Helps facilitate re-methylation
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Why might Homocysteine Accumulate when Vitamin B12 Levels are low? What are high Homocysteine Levels a risk factor for?
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-Vitamin B12 is required to convert Homocysteine to Methionine
-Heart Disease and Stroke |
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What is the Inactive form of THF? How is it activated?
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-Folate
-Reduced |
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Which enzyme catalyzes the activation of Folate to form THF?
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Dihydrofolate Reductase (DHFR)
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What does Dihydrofolate Reductase (DHFR) do? What results?
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Converts Folate (Inactive) to THF (Active)
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What are 2 Important Reactions using Vitamin B12?
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-Resynthesis of Methionine from Homocysteine
-Conversion of Methylmalonyl CoA to Succinyl CoA |
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Describe the Conversion of Methylmalonyl CoA to Succinyl CoA.
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-Vit B12 Derivative is used
-Propionyl CoA from Valine, Isoleucine, or Methionine -Propionyl CoA is Carboxylated using Biotin to give Methylmalonyl CoA -Methylmalonyl CoA converted to Succinyl CoA with help of B12 |
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Which Alpha-Keto Acids can be converted to Nonessential AAs? Which AAs do they become?
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-Pyruvate - Alanine
-Oxaloacetate - Asparatate -Alpha-Ketogluturate - Glutamate |
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How the fuck do we synthesize Tyrosine? What does it come from? What catalyzes this reaction? What cofactors are involved?
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-Phenylalanine
-Phenylalanine Hydroxylase -Tetrahydrobipterin |
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What is Maple Syrup Disease? What is assoicated with? Why does it result?
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-Partial or Complete Deficiency of Alpha-Keto Acid Dehydrogenase: can't break down Branched Chain Acids
-Associated with Isoleucine, Valine, Leucine -Branched AAs accumulate in Plasma and Urine |
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What effects result from Maple Syrup Urine Disease?
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-Neurological Problems
-MR -Physical Disabilities -Death |
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What happens in Maple Syrup Disease? Why? Why is this bad?
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-Branched AAs accumulate in Plasma and Urine
-Deficiency of Alpha-Keto Acid Dehydrogenase: can't break down Branched Chain Acids -Displace NTs in the Brain and Block the TCA Cycle |
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Why is the accumulation of Branched Chain AAs in MSUD a problem?
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-Displace NTs in the Brain
-Block the TCA Cycle |
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What is the treatment for MSUD? Why?
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-Limit Intake of Branched Chain AAs (Isoleucine, Leucine, Valine)
-Can't eliminate because they are essential |
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What is Homocystinuria? Why does it result? How?
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-Due to a Deficiency in Cystathione Synthase
-Can't Convert Homocysteine to Cystathionine -Pushes Homocysteine towards Methionine -Leads to Accumulation of Homocystein and Methionine |
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What is Homocystineuria bad?
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-Leads to Accumulation of Homocystein and Methionine
-Homocystein is associated with Heart Disease and Stroke |
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What are some ways to treat Homocystinuria?
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-Restriction of Methionine
-Administration of Vitamins B6, B12, and Folate -Betaine |
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How can the Administration of Vitamins B6, B12, and Folate help treat Homocystinuria?
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-B6 - May Help Increase Acitivity of Cystathionine Synthase since it is a coenzyme
-B12 - Can Help Remethylate Homocystein to Methionine |
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What happens in PKU?
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-Phenylalanine Hydroxylase Deficiency
-Phenylalanine Hydroxylase converts Phenylalanine to Tyrosine -Accumulation of Phenylalanine bad for development |
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What are some symptoms associated with PKU?
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Mental Retardation
Failure to Walk or Talk Seizure Failure to Grow Hypopigmentation |
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How is PKU treated?
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-Decrease Phenylalanine Intake
-Administer PAH -Administer BH4 - Cofactor |
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Which reactions require BH4 as a Coenzyme?
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-Tyrosine Synthesis
-Catecholamine Synthesis -Serotonin Syntheis |
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What do all the following reactions have in common?
-Tyrosine Synthesis -Catecholamine Synthesis -Serotonin Syntheis |
They all require BH4 as a coenzyme
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