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Biochem - Protein and AA Metabolism and The Urea Cycle

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
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Question
Answer
show Ammonia  
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What are the essential amino acids?   show
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What are the two possible sources of essential amino acids?   show
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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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What is negative nitrogen balance?   show
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When does negative nitrogen balance occur?   show
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What is positive nitrogen balance?   show
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When does positive nitrogen balance occur?   show
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What is tyrosine synthesized from?   show
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show Tyrosine  
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show Pyridoxal phosphateTetrahydrofolateTetrahydrobiopterin (BH4)  
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show NO!  
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show Alanine  
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show Aspartate  
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show Glutamate  
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show Pyruvate  
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What forms aspartate by transamination?   show
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What forms glutamate by transamination?   show
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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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What is transamination?   show
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show Alpha-ketoglutarate accepts the amino acid in transamination, which causes the formation of glutamate.  
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show Transaminases  
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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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show It enables the same enzymes to be used in amino acid degradation and amino acid synthesis.  
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show changes in concentration.  
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show Pyruvate --> alanine  
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show ALT  
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show Oxaloacetate --> aspartate  
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What enzyme is involved in the oxaloacetate --> aspartate reaction?   show
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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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show It deaminates glutamate, resulting in alpha-ketoglutarate and ammonia.  
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show Liver  
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What enzyme is involved in the deamination of glutamate into alpha-ketoglutarate and ammonia?   show
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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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What enzyme is involved in the conversion:glutamine --> glutamate + NH4+?   show
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What does glutaminase do?   show
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show Transporting ammonia to the liver.  
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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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show CANNOT make glucose.Ultimately degraded to Acetyl CoA or acetoacetate.  
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show Promotes amino acid uptake and protein synthesis.Pure protein meal stiumlates insulin secretion but much less than a carb meal would.  
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What is the effect of cortisol on amino acid metabolism?   show
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show Stimulates uptake of amino acids into the liver.  
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Do insulin, cortisol, and glucagon stimulate the uptake of amino acids into the liver?   show
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show Muscles = provide amino acids for metabolismLiver = uses lots of amino acids in fasted state  
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show State of increased fuel usage.Negative nitrogen balance.Cortisol is a mediator.Ex. defense against infection/wound healing  
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Where does the urea cycle occur?   show
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show CPS1 (carbamoyl phosphate synthase 1)  
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What is the rate limiting step in the urea cycle?   show
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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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show Mitochondria  
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In the urea cycle, after the first two steps, where do the following steps occur?   show
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show arginine fumarate  
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show YES!  
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show YES!  
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What is the rate limiting step of the urea cycle?   show
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show Feed forward process(substrate availability)  
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show YES!  
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What is the role of NAG in the urea cycle?   show
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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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show liver disease  
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How does diet affect BUN?   show
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show Increased ammonia levels in the blood.Caused by liver disease or kidney failure (more urea metabolized by intestinal bacteria = incr. BUN)  
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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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