Metabolism of amino acids

Choose essential amino acids

a) Asp, Glu
b) Val, Leu, Ile
c) Ala, Ser, Gly
d) Phe, Trp
Choose essential amino acids
a) Asp, Glu
b) Val, Leu, Ile
c) Ala, Ser, Gly
d) Phe, Trp
Essential amino acids

1) branched chain: Val, Leu, Ile
2) basic: His, Arg, Lys
3) aromatic: Phe ( Tyr), Trp
4) sulfur-containing: Met ( Cys)
5) other: Thr
10
Choose amino acids from which the
other amino acid can be synthesized in
a human body
a) valine leucine
b) aspartate asparagine
c) phenylalanine tyrosine
d) methionine + serine cysteine
Choose amino acids from which the
other amino acid can be synthesized in
a human body
a) valine leucine leucine is the essential AA
b) aspartate asparagine
c) phenylalanine tyrosine
d) methionine + serine cysteine
Synthesis of
ASPARAGINE
needs glutamine as
NH
2
group donor

(it is not ammonia as
in the Gln synthesis)
The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
The figure is from http://web.indstate.edu/thcme/mwking/amino-acid-metabolism.html (Jan 2007)
Synthesis of Tyr from Phe
The figure is from http://web.indstate.edu/thcme/mwking/amino-acid-metabolism.html (Jan 2007)
Synthesis of Cys from Met and Ser
The amino acids can be formed from the
citrate cycle intermediates
in a human body
a) -ketoglutarate glutamate
b) succinyl-CoA isoleucine
c) oxaloacetate aspartate
d) malate threonine
The amino acids can be formed from
the citrate cycle intermediates
in a human body
a) -ketoglutarate glutamate
b) succinyl-CoA isoleucine Ile is the essential AA
c) oxaloacetate aspartate
d) malate threonine Thr is the essential AA
The figure is from
http://www.tcd.ie/Biochemistry/IUBMB-Nicholson/gif/13.html (Dec 2006)
Amphibolic
character
of citrate cycle
The compound(s) can be synthesized
from the amino acid
a) tyrosine serotonin
b) serine ethanolamine
c) tryptophan catecholamines
d) cysteine taurine
The compound(s) can be synthesized
from the amino acid
a) tyrosine serotonin Tyr catecholamines
b) serine ethanolamine formed by decarboxylation
c) tryptophan catecholamines Trp serotonin
d) cysteine taurine
The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
taurin is used in conjugation reactions in the liver
it is bound to hydrophobic substances to increase their solubility
(e.g. conjugation of bile acids)
If the amino acid is metabolised the
substance is formed:
a) methionine gives homocysteine
b) serine gives glycine and folic acid
derivative: methylene tetrahydrofolate
c) glutamine releases ammonia
d) some amino acides can be degraded to
acetoacetate
If the amino acid is metabolised the
substance is formed:
a) methionine gives homocysteine
b) serine gives glycine and folic acid
derivative: methylene tetrahydrofolate
c) glutamine releases ammonia
d) some amino acides can be degraded to
acetoacetate = one of ketone bodies
The figure is from http://web.indstate.edu/thcme/mwking/amino-acid-metabolism.html (Jan 2007)
B12
Regeneration of
Met
(vitamins: folate+B
12
)
The figure is from http://www.biocarta.com/pathfiles/GlycinePathway.asp (Jan 2007)
Synthesis of serine and glycine
glycolysis
Choose products of the transamination
reactions
a) alanine pyruvate
b) glutamate 2-oxoglutarate
c) aspartate oxaloacetate
d) phenylalanine tyrosine
Choose products of the transamination
reactions
a) alanine pyruvate
b) glutamate 2-oxoglutarate
c) aspartate oxaloacetate
d) phenylalanine tyrosine it is not transamination
The figure is from http://web.indstate.edu/thcme/mwking/nitrogen-metabolism.html (Jan 2007)
Transamination reaction
! REVERSIBLE !
enzymes: amino transferases
coenzyme: pyridoxal phosphate (vit. B6 derivative)
The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
alanine aminotransferase
(ALT = GPT)
aspartate aminotransferase
(AST = GOT)
Amino transferases important in medicine
(transaminases)
Amino nitrogen released from carbon
sceletons of AAs can be transported in
blood as
a) NH
4
+
b) alanine
c) glutamine
d) urea
Amino nitrogen released from carbon
sceletons of AAs can be transported in
blood as
a) NH
4
+

physiologically up to 35 mol/l

(NH
3
+ H
+
NH
4
+
)

b) alanine formed by transamination from pyruvate
c) glutamine the most important transport form of NH
2

d) urea it is the end product of degradation of amino nitrogen
(liver kidneys urine)
Transport of
amino nitrogen
from degraded
muscle proteins

products
excreted
with urine
The figure was adopted from Devlin, T. M. (editor): Textbook
of Biochemistry with Clinical Correlations, 4th ed. Wiley-Liss,
Inc., New York, 1997. ISBN 0-471-15451-2
Glucose-alanine cycle
The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
alanine transfers
both the carbon
sceleton for
gluconeogenesis
and NH
2
group
glutamine synthetase
GLUTAMINE
= the most important
transport form af amino
nitrogen in blood



it transfers two amino
groups released by
degradation of AAs

The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
Choose glucogenic amino acids
a) alanine
b) lysine
c) leucine
d) glutamine
Choose glucogenic amino acids
a) alanine
b) lysine
c) leucine
d) glutamine
7 degradation products of AAs
1. pyruvate Gly, Ala, Ser, Thr, Cys, Trp
2. oxaloacetate Asp, Asn
3. -ketoglutarate Glu, Gln, Pro, Arg, His
4. succinyl-CoA Val, Ile, Met, Thr
5. fumarate Phe, Tyr
6. acetyl-CoA Ile
7. acetoacetyl-CoA Lys, Leu, Phe, Tyr, Trp
glucogenic AAs
ketogenic AAs
Glutamate dehydrogenase (GMD)
a) catalyzes conversion of Glu to oxaloacetate
b) is found in mitochondria of hepatocytes
c) produces ammonia
d) needs pyridoxal phosphate as a coenzyme
Glutamate dehydrogenase (GMD)
a) catalyzes conversion of Glu to oxaloacetate
b) is found in mitochondria of hepatocytes
c) produces ammonia
d) needs pyridoxal phosphate as a coenzyme
The figure is from http://web.indstate.edu/thcme/mwking/nitrogen-metabolism.html (Jan 2007)
GLUTAMATE DEHYDROGENASE
removes amino group from carbon sceleton of Glu in the liver
1. NH
2
from AAs was transfered by transamination Glu
2. free ammonia is released by oxidative deamination of Glu
Choose correct statement(s) about
metabolism of amino acids
a) alanine aminotransferase (ALT) transforms
pyruvate to alanine
b) aspartate aminotransferase (AST) transforms
aspartate to -ketoglutarate
c) glutamine synthetase transforms glutamate to
glutamine
d) glutaminase catylyzes conversion of glutamine
to ammonia and -ketoglutarate
Choose correct statement(s) about
metabolism of amino acids
a) alanine aminotransferase (ALT) transforms
pyruvate to alanine
b) aspartate aminotransferase (AST)
transforms aspartate to -ketoglutarate
c) glutamine synthetase transforms glutamate
to glutamine
d) glutaminase catylyzes conversion of
glutamine to ammonia and -ketoglutarate
The figure was adopted from Devlin, T. M. (editor): Textbook of Biochemistry with Clinical Correlations, 4th ed.
Wiley-Liss, Inc., New York, 1997. ISBN 0-471-15451-2
alanine aminotransferase
(ALT = GPT)
aspartate aminotransferase
(AST = GOT)
Amino transferases important in medicine
(transaminases)
Glutamine is principal
transport form of amino nitrogen
The figure is from http://www.sbuniv.edu/~ggray/CHE3364/b1c25out.html (Dec 2006)
The amino acids can enter the citrate
cycle as the molecules
a) alanine acetyl-CoA
b) aspartate oxaloacetate
c) valine succinyl-CoA
d) glutamine -ketoglutarate
The amino acids can enter the citrate
cycle as the molecules
a) alanine acetyl-CoA
b) aspartate oxaloacetate
c) valine succinyl-CoA
d) glutamine -ketoglutarate
The figure is from http://www.biocarta.com/pathfiles/glucogenicPathway.asp (Jan 2007)
The entrance of amino acids into the citrate cycle
Ornithine cycle
a) proceeds only in the liver
b) produces uric acid
c) includes arginine as an intermediate
d) produces energy in a form of ATP
Ornithine cycle
a) proceeds only in the liver
b) produces uric acid
c) includes arginine as an intermediate
d) produces energy in a form of ATP
The figure is from http://www.biocarta.com/pathfiles/ureacyclePathway.asp (Jan 2007)
Detoxication of ammonia in the liver
The figure is from http://courses.cm.utexas.edu/archive/Spring2002/CH339K/Robertus/overheads-3/ch18_TCA-Urea_link.jpg
(Jan 2007)
Interconnection of the urea cycle with the
citrate cycle
In the urea synthesis
a) ammonia reacts with ornithine citrulline
b) carbamoyl phosphate synthetase I (=
mitochondrial) regulates the cycle
c) aspartate is used as a NH
2
group donor
d) urea is formed it can be used as an energy
substrate for extrahepatic tissues
In the urea synthesis
a) ammonia reacts with ornithine citrulline
b) carbamoyl phosphate synthetase I (=
mitochondrial) regulates the cycle
c) aspartate is used as a NH
2
group donor
d) urea is formed it can be used as an energy
substrate for extrahepatic tissues
regulatory enzyme activation inhibition
carbamoyl phosphate
synthetase I
(= mitochondrial)

N-acetylglutamate
N-acetylglutamate
synthetase
arginine
Regulation of urea cycle
allosteric regulation + enzyme induction by protein rich diet or
by metabolic changes during starvation
Urea synthesis is inhibited by acidosis
HCO
3
-
is saved