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HUCM Amino Acids 2
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48
Medical
Graduate
08/23/2011

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Term
What is synthesized by alpha ketoglutarate, ammonia, and NADPH. With glutamate dehydrogenase as the enzyme.
Definition
Glutamate
Term
What is synthesized by glutamate, ammonia, ATP, and glutamine synthetase?
Definition
Glutamine
Term
What is synthesized by oxaloacetate, glutamate and aminotransferase?
Definition
Aspartate
Term
What is synthesized by aspartate, ATP, glutamine, and asparagine synthetase?
Definition
Asparagine
Term
What is synthesized by pyruvate, glutamate, and aminotransferase?
Definition
Alanine
Term
What is synthesized by the urea cycle argininosuccinate, and argininosuccinate lyase?
Definition
Arginine
Term
What is cysteine derived from?
Definition
Serine and the "S" is from Methionine
Term
What is tyrosine derived from?
Definition
Phenylalanine
Term
What is a shared intermediate for both ornithine and proline?
Definition
Glutamic semialdehyde
Term
What can be used as an indicator of commencement of cell division?
Definition
Ornithine Decarboxylase (ODC)
Term
What is the starting material for serine synthesis?
Definition
3-phosphoglycerate
Term
Serine is the starting material for ? and ?
Definition
cysteine and glycine
Term
What is a point of entry for serine carbons directly into the gluconeogenic pathway?
Definition
3-phosphoglycerate
Term
Serine + THF produces what?
Definition
N5, N10- methyleneTHF + glycine (biosynthesis of glycine)
Term
THF (tetrahydrofolate) consists of 3 groups: A ?, an amino benzoate and a chain of one or more glutamate residues.
Definition
pteridine
Term
THF (tetrahydrofolate) consists of 3 groups: A ?, an amino benzoate and a chain of one or more glutamate residues.
Definition
pteridine
Term
Folate is reduced by dihydrofolate reductase to form ? This requires what type of energy
Definition
THF
NADPH
Term
What allows for the transfer of one-carbon groups from a donor to a recipient that needs the carbon at a different oxidation level?
Definition
THF
Term
There is another carrier of one-carbon groups called ?, it can carry the carbon ONLY as a methyl group, so it is not as versatile as THF.
Definition
SAM (AdoMet)
Term
How many reactions are there where the reducing power comes from the double bonds of the THF itself.
Definition
ONE
Term
Degradation of serine to 3-phosphoglycerate would be a very effective means of using serine carbons for ?
Definition
gluconeogenesis
Term
What is the 21st amino acid coded for in mammalian DNA?
What is the starting material?
Definition
Selenocysteine
Serine
Term
Selenocysteine can be synthesized ONLY when serine is esterified to a ?
Definition
tRNA (specifically the tRNA that bears the anticodon 5'-UCA-3'
Term
Serine can serve as a headgroup for ?
Serine can be decarboxylated to ?
Definition
phosphoglycerides
ethanolamine
Term
The reaction of serine to glycine uses what enzyme?
What is this reaction dependent on?
Definition
serine hydroxymethyltransferase
THF
Term
In vivo the function of the glycine cleavage enzyme is only in ? of glycine.
Definition
degradation
Term
Is threonine ketogenic or glucogenic?
Definition
BOTH
Term
If there is a defect in either of the two enzymes that convert Phenylalanine to Tyrosine (which are ?? amd ??)this is a cause for what disease?
Definition
Phenylalanine hydroxylase and DHB reductase
PKU (phenylketonuria)
Term
If there is a deficiency in what precursor can cause PKU in the reaction of phenylalanine to tyrosine
Definition
GTP (Tetrahydrobiopterin)
Term
With the reaction of tyrosine to homogentisate if there is a deficiency in tyrosine transaminase what disease results?
Definition
Tyrosinemia 2
Term
When going from homogentisate to maleylacetoacetate a deficiency in homogentisate oxidase will result in what disease?
Definition
Alkaptonuria
Term
When going from maleylacetoacetate to fumarylacetate if there is a deficiency it will result in ?
Definition
Tyrosinemia 1
Term
Are Phe and Tyr glucogenic or ketogenic?
Definition
BOTH
Term
If there is a defective rxn going going from fumarylacetate to whatever follows it what diease is the result?
Definition
Tyrosinemia 1
Term
The combination of methionine ATP and methionine adenosyltransferase results in what?
Definition
SAM (AdoMe a.k.a. S-adenosylmethionine)
Term
What is a one-carbon longer analog of pyruvate?
Definition
alpha-ketobutyrate
Term
An enzyme metabolizes alpha ketobutyrate to ? ?
Definition
propionyl CoA
Term
What can also directly produce alpha ketobutyrate?
Definition
Homocysteine
Term
What to know about tryptophan?
1. The metabolites that can enter intermediary metabolism are alanine, and ? ?, which is a further metabolite of glutarate.
Definition
Acetoacetyl CoA
Term
In regards to tryptophan the first enzyme in the pathway is ? ?-?)
Definition
tryptophan di-oxygenase
Term
The reaction of tryptophan and O2 results in ?
Definition
N-formylkynurenine
Term
Tryptophan is eventually converted into ?
Definition
glutarate and then to acetoacetyl CoA
Term
What three amino acids are considered the branch chain amino acids?
Definition
valine, leucine, and isoleucine
Term
Valine, leucine, and isoleucine provide a large amount of energy for ?
Their metabolic pathways produce a lot of ?
Definition
muscle
NADH
Term
Valine produces ?
Definition
Propionyl CoA
Term
Isoleucine produces ? ? and ? ?
Definition
Propionyl CoA and Acetyl CoA
Term
Leucine (ketogenic) makes ?
Definition
acetoacetate
Term
Lysine's final product is ?
Definition
Acetoacetyl CoA
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