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BASIC STRUCTURES
Abbr
ev.
Full
Name
A Ala Alanine
hydrophobic
C Cys Cysteine
hydrophobic
(Nagano, 1999)
Remarks
Very abundant and versatile. It behaves fairly
neutrally and can be located in both
hydrophilic regions on the outside of the
protein and hydrophobic interior areas.
The sulfur atom binds readily to heavy metal
ions. Under oxidizing conditions, two cysteines
can be joined together by a disulfide bond to
form the amino acid cystine. When cystines
are components of a protein, they enforce
tertiary structure and makes the protein more
resistant to unfolding and denaturation;
disulfide bridges are therefore common in
proteins that have to function in harsh
environments, digestive enzymes (e.g., pepsin
and chymotrypsin), structural proteins (e.g.,
keratin), and proteins too small to hold their
shape on their own (eg. insulin).
Behaves similarly to glutamic acid. Carries a
hydrophilic acidic group with strong negative
charge. Usually is located on the outer surface
of the protein, making it water-soluble. Binds
to positively-charged molecules and ions,
Abbrev Full
.
Name
Side
chain
type
Remarks
Abbre
v.
*K Lys
*L Leu
*
Met
M
N Asn
Full
Name
Side chain
type
Remarks
Abbre
v.
Full
Name
P Pro Proline
Q Gln
Side chain
type
Remarks
Glutamin
Neutralized version of glutamic acid. Used in proteins
hydrophilic
e
and as a storage for ammonia.
*R Arg Arginine
Basic
S Ser Serine
Abbrev.
Full
Name
Side chain
type
Remarks
*T Thr
Threonine hydrophilic
*V Val
Valine
Behaves similarly to
leucine. See isoleucine.
*W Trp
Tyr
Tyrosine
hydrophobic
isoleucine
and
GENERALITIES
Classification
Neutral
Acidic
Basic
Neutral
Aliphatic
Aromatic
Heterocyclic
S-containing AA
Generalities - neUTRAL
A.
Aliphatic
1.
2.
3.
4.
5.
generalities
B. Aromatic
8. Phenylalanine
(Phe) (F) or alpha
amino phenyl
propionic acid
C. Heterocyclic AA
10. Tryptophan (Trp) (W)
alpha amino 3
indole propionic
- often considered as
aromatic AA since it has
aromatic ring in its
structure.
11. Histidine (His) (H)
alpha amino imidazole propionic acid
Histidine is basic in
solution on account of the
imidazole ring and often
considered as Basic AA
generalities
D. Imino Acids
12. Proline (Pro) (P) or
Pyrrolidone 2 carboxylic
Acid
13. Hydroxyproline (Hyp) or
4 Hydroxy pyrrolidone 2
carboxylic acid
Proline & Hydroxyproline
do not have a free _NH2
group but only a basic
pyrrolidone ring in which the
Nitrogen of the Imino group
is in ring but can still
function in the formation of
peptides.
Are called Imino Acids.
- contains 2 sulfur
containing AA
14. Cysteine (Cys) (C) or
alpha amino
mercaptopropionic acid
S S linkage is called a
Disulfide bridge
15. Methionine (Met) (M) or
alpha amino y methylthio - butyric acid
GENERALITIES - ACIDIC
II. Acidic AA
- monoaminodicarboxylic Acids
GENERALITIES - BASIC
III. Basic AA
GENERALITIES
Classification of Amino Acids
based on polarity
of the R group:
4 groups
Polarity reflects the functional
role
of AA in protein
structure
GENERALITIES
1. Non-polar AA
hydrophobic (water hating)
No charge on the R group
Examples are:
Alanine Methionine
Leucine Phenylalanine
Isoleucine
Tryptophan
Valine
Proline
GENERALITIES
Histidine
Arginine
Lysine
Glutamic Acid
Aspartic Acid
GENERALITIES
on R group
no charge on the R group
possess groups
hydroxyl
sulfhydryl
amide
participate in hydrogen bonding of
protein structure
Examples:
Asparagine Glycine
Cysteine
Tyrosine Serine
Threonine
Glutamine
GENERALITIES
Zwitter
from German word means
hybrid
GENERALITIES
GENERALITIES
GENERALITIES
Tryptopha
n
Histidine Arginin
e
Valine
Phenylalanin
e
PV
T
Threonine
TI
M
HAL
L
Leucine
Lysine
Methionin
e
Isoleucine
Essential Amino
Acids
Ca
r
carnitine -->
butyrobetaine -->
Lysine,
Vitamin
methioni
C
ne
fat
metabolism
Ornithin
zinc
e
urea cycle
Citrullin
e
Cit -
argenine -->
citrulline -->
ornithine
Hydroxy
proline
Hy
p
proline -->
hydroxyproline
Vitamin
C
collagen
Ornithin
e
Or
n
argenine -->
citrulline -->
ornithine
urea cycle
- Taurine -
Ta
u
methionine -->
GABA,
cysteine --> taurine glycine
B6
heart, bile
The above are precursors for, or important products of, the 20 "true"
amino acids listed above.
NON-ESSENTIAL
Transamination of -KETOACIDS that are
available as common intermediates
ESSENTIAL
Their -KETOACIDS are not common
intermediates (Enzymes needed to form
them are lacking)
Amino Acids
A
m
i
n
o
A
c
i
d
M
e
t
a
b
o
l
i
s
m
Glucogenic
Glucogenic
and Ketogenic
Ketogenic
Non-Essential
AA
Alanine
Asparagine
Aspartate
Cysteine
Glutamate
Glutamine
Glycine
Proline
Serine
Tyroxine
Essential AA
Arginine
Histidine
Methionine
Threonine
Valine
Isoleucine
Phenylalanine
Tryptophan
Leucine
Lysine
O
IN
AM
ID
C
A
Y
C
EN ISM
I
F
IC O L
F
O
E
S
D TAB
R
E
E M
E
D
M
M
S
R LI ZY D
O
I
IS BO EN AC
D TA C
D
E IFI INO
E
M C M
IT
R
PE A
E
S
F
H
:
O
Y
N
IT RS
,I
L
E
A
O
R
R
A
M
R
R ER
O
N
N
B
R
A
O
B
IN
aminoacidopathies
TYROSINEMIA
ALKAPTONURIA
PHENYLKETONURIA
DEFECTIVE PROCESS: CONVERSION OF
PHENYLALANINE TO TYROSINE
DEFECTIVE ENZYME: PHENYLALANINE
HYDROXYLASE (Phenylalanine-4-mono-oxygenase)
An autosomal recessive trait urine has a musty
odor
Compounds seen in both urine and blood:
Phenylpyruvic acid primary catabolite
Phenyllactic acid product of deaminzation
Phenylacetylglutamine produced from oxidation
of phenylpyruvic acid
PHENYLKETONURIA
DEFECTIVE PROCESS: METABOLISM OF THREE ESSENTIAL BRANCHEDCHAINAMINO ACIDS (LEUCINE, ISOLEUCINE AND VALINE)
DEFECTIVE ENZYME: BRANCHED CHAIN -KETO ACID DECARBOXYLASE
COMPLEX
Burnt sugar odor of urine, breath and skin
Screening: Modified Guthrie test metabolic inhibitor of B. subtilis
which is 4-azaleucine is impregnated in the medium
POS (+) for MSUD = bacterial growth
Microfluorometric assay using leucine dehydrogenase
Filter paper specimen is treated with methanol and acetone to
denature hemoglobin
The NADH fluorescence produced is measured at 450nm; excitation
wavelength at 360nm
Confirmed diagnosis is based on finding increased plasma and urinary
levels of the three branched-chain amino acids and their ketoacids with
LEUCINE (highest: above 4mg/dL)
Prenatal diagnosis: measuring decarboxylase enzyme concentration in
cells cultured from amniotic fluid
CYSTINURIA
ARGININOSUCCINIC ACIDURIA
AND PROCESS:
CITRULLINEMIA
DEFECTIVE
UREA CYCLE
DEFECTIVE ENZYME: ARGININOSUCCINIC
ACID LYASE
Decrease in activity of ASA synthetase
Citrullinemia
Citrulline is elevated in MS/MS
Argininosuccinic aciduria milder
elevations
Citrullinemia dramatic elevations
* Ornithine and arginine are seen
increased in older infants
Isovaleric acidemia