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Ch. 30.1-30.3
Ch. 30.1-30.3
125
Biochemistry
Undergraduate 3
02/24/2018

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Term
some sources of amino acids
Definition
-dietary protein
-degradation of unneded or defective cellular proteins
Term
protein turnover
Definition
the degradation and resynthesis of proteins
Term
some proteins that are short lived
Definition
those that are involved in metabolic regulation
Term
some causes of defective proteins
Definition
-errors in translation
-oxidative damage
-altered in other ways with the passage of time
Term
something that happens to excess amino acids
Definition
they are first used as building blocks for anabolic rxns, such as protein synthy and nucleotide synth
Term
what happens to excess amino acids after then needs for anabolic rxns are met?
Definition
the amino acids are degraded and the C skeletons are used in catabolism or anabolism
Term
why it's important to safely remove the excess N from amino acids
Definition
because excess N in the form of ammonia is toxic
Term
how most vertebrates get rid of excess N from amino acids
Definition
convert the ammonia into urea, which is then excreted
Term
the first step in the degradation of amino acids
Definition
N removal
Term
the major site of amino acid degradation in mammals
Definition
liver
Term
α-ketoacids
Definition
what's left over after the N is removed from amino acids
Term
what happens after the N is removed fro amino acids?
Definition
the resulting α-ketoacids so that the C skeletons can enter the metabolic mainstream as precursors of glucose or citric acid cycle intermediates
Term
how alpha-amino groups are converted into ammonium ions
Definition
by the oxidative deanimation of glutamate
Term
depiction of oxidative deanimation of glutamate
Definition
[image]
Term
aminotransferases aka transaminases
Definition
catalyze the transfer of an α-amino groupfrom an α-amino acid to an α-ketoacid

generally funnel α-amino groups from a variety of amino acids to α-ketoglutarate for conversion into ammonia
Term
depiction of the function of aminotransferases aka transaminases
Definition
[image]
Term
aspartate aminotransferase
Definition
catalyzes the transfer of the aminotransferases aka transaminases-amino group of aspartate to aminotransferases aka transaminases-ketoglutarate
Term
depiction of the function of aspartate aminotransferase
Definition
[image]
Term
alanine aminotransferase
Definition
catalyzes the transfer of the amino group of alanine to aminotransferases aka transaminases-ketoglutarate
Term
depiction of the function of alanine aminotransferase
Definition
[image]
Term
depiction of the oxidative deanimation of glutamate, regenerating α-ketoglutarate
Definition
[image]
Term
glutamate dehydrogenase
Definition
catalyzes the oxidative deanimation of glutamate
Term
depiction of the function of glutamate dehydrogenase
Definition
[image]
Term
something unusual about glutamate dehydrogenase
Definition
it can use either NADH or NADPH as reducing power
Term
how toxic free ammonia is sequestered
Definition
some liver specific enzymes in mitochondria such that there's compartmentalization
Term
how glutamate dehydrogenase is regulated in mammals, but not other organisms
Definition
allosterically inhibited by GTP and stimulated by ADP
Term
arborative complex
Definition
this is formed on an enzyme when a product is replaced by substrate before the rxn is complete
Term
the net rxn catalyzed by aminotransferases and glutamate dehydrogenases
Definition
α-amino acid + NAD(P)H+ + H2O --> α-ketoacid + NH4+ + NAD(P)H + H+
Term
what happens to the N atoms of amino acids, except for serine and threonine, before their removal?
Definition
they get transferred to α-ketoglutarate before their removal
Term
how the N atoms of serine and threonine are removed
Definition
they are directly deanimated
Term
serine dehydratase
Definition
catalyze the direct deanimation of serine, which yields pyruvate and ammonia
Term
threonine dehydratase
Definition
catalyze the direct deanimation of thrionine, which yields α-ketobutyrate and ammonia
Term
the prosthetic group on serine dehydratase and thrionine dehydratase
Definition
pyridoxal phosphate (PLP)
Term
depiction of the function of serine dehydratase
Definition
[image]
Term
depiction of the function of threonine dehydratase
Definition
[image]
Term
depiction of how ammonia is converted into urea in most terrestrial vertibrates
Definition
[image]
Term
the amino acids liver can not deanimate
Definition
the branched-chain amino acids leucine, valine, and isoleucine
Term
one way muscle uses branched-chain amino acids
Definition
as a source of fuel
Term
how N from amino acids is processed in the muscle
Definition
gets released into a form that can be absorbed by the liver and converted into urea
Term
2 principal transport forms in which N is transported from muscle to liver
Definition
-alanine
-glutamine
Term
glucose-alanine cycle
Definition
evidently the interconversion of alanina and glucose
Term
depiction of the glucose-alanine cycle
Definition
[image]
Term
when muscles use branched-chain amino acids as fuel
Definition
during prolonged exercise and fasting
Term
a key transport form of N
Definition
glutamine
Term
glutamine synthetase
Definition
catalyzes the synthesis of glutamine from glutamate and ammonia in an ATP-dependent rxn
Term
depiction of the function of glutamine synthetase
Definition
[image]
Term
how ammonia from the breakdown of amino acids can be used by the organism
Definition
in the biosynthesis of N compounds
Term
what happens to excess ammonia in most terrestrial vertebrates?
Definition
gets converted to urea by the urea cycle and excreted
Term
ureotelic organisms
Definition
organisms that use the urea cycle to convert excess ammonia into urea to ne excreted
Term
depiction of the urea cycle
Definition
[image]
Term
structure of urea
Definition
[image]
Term
urea cycle begins in ______ with ______
Definition
mitochondrial matrix coupling of free NH4+ and HCO3- to form carbamoyl phosphate
Term
carbamoyl phosphate synthetase I (CPS I)
Definition
catalyzes the coupling of free NH4+ and HCO3- to form carbamoyl phosphate
Term
the committed step in the urea cycle
Definition
the coupling of free NH4+ and HCO3- to form carbamoyl phosphate using carbamoyl phosphate synthetase I (CPS I)
Term
depiction of the rxn sequence that forms carbamoly phosphate
Definition
[image]
Term
why the synthesis of carbamoyl phosphate is irreversible
Definition
because it consumes 2 ATP's
Term
the key regulatory enzyme for urea synthesis
Definition
carbamoyl phosphate synthetase
Term
when carbamoyl phosphate synthetase is maximally active
Definition
when amino acids are being metabolized for fuel use
Term
N-acetylglutamate synthase
Definition
catalyzes the synthesis of N-acetylglutamate
Term
depiction of the function of N-acetylglutamate synthase
Definition
[image]
Term
N-acetylglutamate synthase is itself activated by...
Definition
arginine
Term
how the urea cycle begins
Definition
carbamoyl phosphate reacts with ornithine to begin the urea cycle
Term
ornithine transcarbamoylase
Definition
catalyzes the rxn of ornithine with carbamoyl phosphate to form citrulline
Term
depiction of the function of ornithine transcarbamoylase
Definition
[image]
Term
argininosuccinate synthetase
Definition
catalyzes the formation of argininosuccinate from citrulline and aspartate
Term
depiction of the function of argininosuccinate synthetase
Definition
[image]
Term
argininosuccinase aka argininosuccinate lyase)
Definition
cleaves argininosuccinate into arginine and fumarate
Term
depiction of the function of argininosuccinase aka argininosuccinate lyase
Definition
[image]
Term
arginase
Definition
catalyzes the hydrolysis of arginine to form urea and ornithine
Term
the net rxn of urea synthesis
Definition
CO2 + NH4+ + 3 ATP + aspartate + 2 H2O --> urea + 2 ADP + 2 Pi + AMP + PPi + fumarate
Term
importance of fumarate derived from urea cycle
Definition
-fumarate is a precursor for glucose
-fumarate can be hydrated to malate, which is an intermediate in the citric acid cycle
Term
the urea cycle, citric acid cycle, and transamination of oxaloacetate are linked by...
Definition
fumarate and aspartate
Term
depiction of the connection between the urea cycle, the citric acid cycle, and the transamination of oxaloacetate
Definition
refer to p. 557
Term
the strategy of amino acid degradation
Definition
to transform the C skeletons into major metabolic intermediates that can be converted into glucose or oxidized by the citric acid cycle
Term
the C skeletons from the 20 fundamental amino acids are funneled into these 7 molecules.
Definition
-pyruvate
-acetyl CoA
-acetoacetyl CoA
-α-ketoglutarate
-succinyl CoA
-fumarate
-oxaloacetate
Term
ketogenic amino acids
Definition
amino acids that are degraded into acetyl CoA or acetoacetyl CoA

they are called this because they give rise to ketone bodies or fatty acids but can not be used to synthesize glucose
Term
ketogenic amino acids give rise to...
Definition
ketone bodies or fatty acids
Term
ketogenic amino acids give rise to ketone bodies or fatty acids but can not be used to synthesize...
Definition
glucose
Term
glucogenic amino acids
Definition
amino acids that are degraded to pyruvate, α-ketoglutarate, succinyl CoA, fumarate, or oxaloacetate
Term
what oxaloacetate and pyruvate derived from the degradation of glucogenic amino acids can be used for
Definition
gluconeogenesis
Term
the only 2 amino acids that are solely ketogenic
Definition
-leucine
-lysine
Term
the amino acids that are both ketogenic and glucogenic
Definition
-threonine
-isoleucine
-phenylalanine
-tryptophan
-tyrosine
Term
the amino acids that are solely glucogenic
Definition
-alanine
-cysteine
-glycine
-serine
-threonine
-asparagine
-aspartate
-methionine
-valine
-arginine
-glutamate
-glutamine
-histidine
-proline
Term
how the poathways of amino acid degradation are identified
Definition
by entry point into metabolism
Term
depiction of the fates of the C skeletons of amino acids
Definition
[image]
Term
amino acids that enter the metabolic mainstream by pyruvate
Definition
-alanine
-serine
-cysteine
-glycine
-threonine
-tryptophan
Term
how alanine gets converted into pyruvate
Definition
transamination of alanine

alanine + α-ketoglutarate <--> pyruvate + glutamate
Term
how serine gets converted into pyruvate
Definition
deanimation by dehydratase serine --> pyruvate + NH4+
Term
serine dehydratase
Definition
catalyzes the deanimation of serine to pyruvate
Term
depiction of the function of serine dehydratase
Definition
[image]
Term
depiction of pyruvate formation from amino acids
Definition
[image]
Term
the amino acids that enter the mainstream metabolism by way of oxaloacetate
Definition
-asparagine
-aspartate
Term
how aspartate enters mainstream metabolism by way of oxaloacetate
Definition
gets directly transaminated to oxaloacetate

aspartate + α-ketoglutarate <--> oxaloacetate + glutamate
Term
how asparagine enters mainstream metabolism by way of oxaloacetate
Definition
gets hydrolyzed into aspartate, which is then transaminated into oxaloacetate
Term
asparaginase
Definition
catalyzes the hydrolysis of asparagine into aspartate and NH4+
Term
amino acids that can enter mainstream metabolism by α-ketoglutarate
Definition
-glutamine
-arginine
-proline
-histidine
-glutamate
Term
how glutamate enters mainstream metabolism by way of α-ketoglutarate
Definition
gets converted into α-ketoglutarate
Term
how glutamine, proline, arginine, and histidine enter mainstream metabolism by way of α-ketoglutarate
Definition
by getting converted into glutamate, which can be converted into α-ketoglutarate
Term
depiction of α-ketoglutarate formation from amino acids
Definition
[image]
Term
depiction of histidine degradation (the conversion of histidine into glutamate)
Definition
[image]
Term
how glutamine enters mainstream metabolism by way of α-ketoglutarate
Definition
gets hydrolyzed into glutamate, which can be converted into α-ketoglutarate
Term
glutaminase
Definition
hydrolyzes glutamine into glutamate and NH4+
Term
depiction of glutamate formation from proline and arginine
Definition
[image]
Term
amino acids that enter mainsteram metabolism by way of succinyl CoA
Definition
-methionine
-isoleucine
-valine
Term
depiction of succinyl CoA formation from methionine, isoleucine, and valine
Definition
[image]
Term
the branched chain amino acids that enter mainstream metabolism
Definition
-leucine
-isoleucine
-valine
Term
branched-chain α-ketoacid dehydrogenase complex
Definition
catalyzes the oxidative decatboxylation of α-ketoisocaproate into isovaletyl CoA
Term
depiction of leucine being converted into isovaleryl CoA
Definition
[image]
Term
depiction of the function of the branched-chain α-ketoacid dehydrogenase complex
Definition
[image]
Term
depiction of isovaleryl CoA being converted into β-methylglutaconyl CoA
Definition
[image]
Term
depiction of β-methylglutaconyl CoA being converted into acetyl CoA and acetoacetate
Definition
[image]
Term
overall conversion of leucine into acetyl CoA and acetoacetate
Definition
make a composite picture of the 3 rxn sequences on p. 563
Term
what is used to break the aromatic ring in aromatic amino acids?
Definition
O2
Term
phenylalanine hydroxylase
Definition
catalyzes the hydroxylation of phenylalanine to tyrosine
Term
depiction of the function of phenylalanine hydroxylase
Definition
[image]
Term
monooxygenase or mixed-function oxygenase
Definition
oxygenase that makes 1 O atom of O2 go to the product and the other go to H2O example: phenylalanine hydroxylase
Term
net rxn for the hydroxylation of phenylalanine to tyrosine
Definition
phenylalanine + H2O + NADH + H+ --> tyrosine + NAD+ + H2O
Term
depiction of phenylalanine and tyrosine degradation
Definition
[image]
Term
the end products of phenylalanine and tyrosine degradation
Definition
acetoacetate and fumarate
Term
nearly all cleavages of aromatic rings in biological systems are catalyzed by...
Definition
dioxygenases
Term
dioxygenase
Definition
enzyme that incorporates both O atoms into the product
Term
depiction of tryptophan degradation
Definition
[image]
Term
end products of tryptophan degradation
Definition
alanine and acetoacetate
Term
the end product of methionine degradation
Definition
succinyl CoA
Term
depiction of methionine metabolism
Definition
[image]
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