Biochem - Protein and AA Metabolism and The Urea Cycle
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| AA catabolism produces what toxic product? | show 🗑
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| show | PVT TIM HALL(phenylalanine, valine, tryptophan, threonine, isoleucine, methionine, histidine, arginine, lysine, leucine)
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| show | diet, protein turnover
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| show | Carbon (synthesis of glucose, fatty acids, ketones and energy)Nitrogen (urea)Synthesis of other N-containing molecules (purines, pyrimidines, porphyrine (bilirubin), neurotransmitters)
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| show | When input is less than output. Endogenous amino acids are being broken down, thus "excreting" and is more than is being taken in.
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| show | Dietary Deficiency (starvation)Catabolic stress (ex. infection)
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| show | When input is greater than output. You are taking in more nitrogen than you are excreting.
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| show | Childhood (growth)Pregnancy
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| What is tyrosine synthesized from? | show 🗑
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| What does phenylalanine synthesize? | show 🗑
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| show | Pyridoxal phosphateTetrahydrofolateTetrahydrobiopterin (BH4)
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| Can essential amino acids be synthesized by the body? | show 🗑
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| show | Alanine
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| When oxaloacetate is transaminated, what does it synthesize? | show 🗑
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| show | Glutamate
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| What forms alanine by transamination? | show 🗑
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| show | Oxaloacetate
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| show | alpha-ketoglutarate
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| Where is arginine generated? | show 🗑
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| Where does Cysteine get its S? | show 🗑
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| Can some enzymes used in amino acid synthesis also be used in amino acid degradation? | show 🗑
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| show | Vitamin B6
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| show | TransaminationDeamination
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| What is deamination? | show 🗑
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| show | Where an amino group is transferred to another molecule. This does NOT result in ammonia production. Most cells do this (rather than deamination)
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| What do alpha-ketoglutarate and glutamate do in transamination? | show 🗑
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| What is another name for aminotransferases? | show 🗑
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| What is another name for transaminases? | show 🗑
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| What do transaminases/aminotransferases require as a cofactor? | show 🗑
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| show | YES!
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| Why is it important that transamination be reversible? | show 🗑
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| The direction of a transaminase reaction will reverse in response to ... | show 🗑
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| show | Pyruvate --> alanine
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| What enzyme is involved in the pyruvate --> alanine reaction? | show 🗑
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| show | Oxaloacetate --> aspartate
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| show | AST
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| show | Liver damage, because they are released into the plasma (thus showing up as high levels in the blood). You will also see low albumin levels in the blood, because albumin is the most abundant protein synthesized in the liver.)
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| What is the function of glutamate dehydrogenase? | show 🗑
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| show | Liver
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| show | glutamate dehydrogenase
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| show | Glutamine synthase
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| show | glutamate and ammonium --> glutamine
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| How many ammonia molecules does glutamine have? | show 🗑
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| show | Glutaminase
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| What does glutaminase do? | show 🗑
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| What transport role is alanine important in? | show 🗑
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| How are pyruvate and ALT involved in the transport of ammonia to the liver? | show 🗑
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| show | Can create glucose.Ultimately degraded to pyruvate or TCA cycle intermediates.
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| What defines ketogenic amino acids? | show 🗑
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| What is the effect of insulin on amino acid metabolism? | show 🗑
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| show | Stimulates uptake of amino acids into the liver.Induces ubiquitin synthesis.Stimulates gluconeogenesis so amino acids are needed to provide precursors.
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| What is the effect of glucagon on amino acid metabolism? | show 🗑
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| show | YES!
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| show | Muscles = provide amino acids for metabolismLiver = uses lots of amino acids in fasted state
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| What is a hypercatabolic state? | show 🗑
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| show | Liver
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| What is the enzyme that converts HCO3- + NH4+ --> Carbamoyl phosphate? | show 🗑
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| show | CPS1 (carbamoyl phosphate synthase 1)
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| show | OTC (ornithine transcarbomylase)
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| Where do the two N's in urea come from? | show 🗑
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| show | Glutamate
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| Where do the first two steps of the urea cycle occur? | show 🗑
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| show | Cytoplasm
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| What are two products from the urea cycle? | show 🗑
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| show | YES!
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| Is the process of urea formation irreversible? | show 🗑
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| What is the rate limiting step of the urea cycle? | show 🗑
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| How is the urea cycle regulated? | show 🗑
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| show | YES!
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| show | It is synthesized by acetyl CoA and glutamate and it STIMULATES CPS1
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| show | Can cross membranes and is filtered into blood.Then, filtered by kidneys and excreted in urine.Some urea diffuses into intestines and is cleaved by bacteria.
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| What is BUN? | show 🗑
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| What does an elevated BUN show? | show 🗑
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| What does a low BUN show? | show 🗑
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| How does diet affect BUN? | show 🗑
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| What is hyperammonemia? | show 🗑
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| Ornithine transcarbamoylase deficiency (OTC), the most common cause of urea cycle disorder, is what type of genetic trait? | show 🗑
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