Structures and pathways Flashcards
Draw glycolysis completely, all products, substrates, enzymes, GAPDH reaction (only this one) and the additional things in the reaction.
Refer to notes.
Draw all the CAC, with inputs of water, NADH, FADH2, etc.
Refer to notes.
Draw the PDC mechanism.
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Draw B-oxidation.
Refer to notes.
Draw the Aspartate-malate shuttle.
Refer to notes.
Draw the generation of ketone bodies.
Refer to notes.
Draw the urea cycle.
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Draw the pathway, from b-hydroxybutyrate for how the body uses ketone bodies.
Refer to notes.
Draw the glucose alanine cycle.
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Draw the urea cycle.
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Draw alanine catabolism.
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Draw Aspargine catabolism
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Draw glutamine catabolism
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Draw Branched chain a-keto acid DH complex pathway.
Refer to notes (same as PDC tbh)
Draw the PPP
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Describe ammonia toxicity.
Two reasons this can happen:
1 - Can get this if the urea cycle is not working properly or
2 - inhale/ingest some form of ammonia.
Ammonia is soluble in the blood and can reach the brain.
Brain tries to get rid of ammonia.
A-keto (common scavenger), takes ammonia and is converted to glutamate by glutamate DH.
Glutamate functions as a NT so it needs to get rid of it and, it cant go in the blood, converted to glutamine using glutamine synthase.
Glutamine is placed in the blood and sent to the liver.
However, this is where the toxicity occurs:
- take lots of a-keto from CAC, gets blocked, not lots of ATP generation in brain
Also, since glutmate is a NT, can create havock, too much is a problem and too little is a problem
- without glutamate, can faint
What are therapies for defects in the urea cycle?
1 - Defined diet with only essential amount of amino acids
2 - Benzoate or phenylacetate - react with glycine and glutamine (respectively) and get excreted in the urine - causes these to be low in body so ammonia levels go down due to synthesis
Which amino acids are branched chain?
Draw R.
Valine (1-3 bis), leucine (2,1,3), isoleucine (1,2,3)
What is a glucogenic amino acid?
Glucogenic aa - catabolized to produce something like pyruvate, oxaloacetate, succinyl-CoA, which can be converted to glucose by neoglucogenesis.
What is a ketogenic amino acids?
Amino acids that make acetyl-CoA when catabolized, converted to ketone bodies.
Describe alkaptonuria.
What process is defective?
What enzyme is defective?
What are the symptoms?
Process: Tyrosine degradation
Enzyme: homogentisate-1,2-dioxygenase
Symptoms: excrete homogentisate
Describe phenylketouria.
What process is defective?
What enzyme is defective?
What are the symptoms?
Process: Conversion of phenylalanine to tyrosine
Enzyme: phenylalanine hydroxylase
Symptom: Excrete Phenylalanine
Describe maple syrup urine disease. What process is defective? What enzyme is defective? What are the symptoms? Treatment?
Process/enzyme: Branched chain keto-acid DHC is defective
Symptoms: brain issues, muscle fatigue, urine smells like maple syrup, branched-chain keto acids accumulate in urine
Treatment: carbs and fats as major dietary source, only small amount of protein for growth
- remove branched chain amino acids from diet
Describe MMA. What process is defective? What enzyme is defective? What are the symptoms? Treatment?
Process: Odd-chain fatty acid catabolism (convert propionyl-A- succinyl-CoA)
Enzyme: Methyl-malonyl-Coa mutase
Symptoms: accumulation of methyl-malonyl-CoA
- fatal, vomiting convulsions, etc.
Treatment: reduce protein intake, remove isoleucine and leucine (branched chain aa) and odd chain fatty acids.
