study method
Metabolism of phenylanine and tyrosine
:
1) Go over learning objectives
.
.
2) Go through slides
-
.
3) klatch a youtube
-
video .
The rate determines if the
limiting step pathway will
4) Read through book
.
5) prepare notes .
and study .
continue , the amount of product that will be formed
etc
.
this if there is an absence of either
•
Discuss the metabolism of Phenylalanine & In case ,
Tyrosine and its related disorders of the highlighted →
Phenylketonuria
As Phy cannot be converted to Tyr ,
instead it
phenyl pyruvate
which in turn becomes
is made into
Phenylalanine PhenylLactate Phenyl acetate Phenylethylamine Phenyl acetyl
glutamine
-
[All are ketogenic]
•
proteins
proteins
make
are made
up most
of
of
amino acids -7
our body → Phenylketonuria [pku]
SIGNS 1- SYMPTOMS
20 amino acids found in the body
{
'
d e l
1 progressive)
including s
'
phenylamine . m m p
.
It is an essential amino acid i. e it
delayed musty se " " " "
to be taken
smell tighter
needs in diet .
development skin
•
Found in amino acid pools in our body eczema Psychiatric disorders
that exist bec proteins don't have a
mental retardation
storage form .
* No symptoms may appear in 1st few months .
Importance precursor for aromatic compounds
Diagnosis
•
:
required for bodily functions .
1) Check plasma phe Levels l may not be
Tyrosine
-
detectable 3-4 days postpartum ] .ie birth
2) An older + less reliable screening test
.
It is a non essential -
aa formed from Phe Uses Felts to detect phenyl pyruvate
and is an intermediate in it 's normal metabolism in Urine .
related to
genetics
→ →
+ degradation pathway .
probes In
* DNA prenatal "
facilitate
of PAH /
diagnosis defects in
Normal Metabolism dihydro biopterin reductase .
catecholamines TYPES :
PAH
[ dopamine epinephrine
Phe
,
Tlff
,
norepinephrine -13 Tu]
, ,
•
Hyper phenylalaninemias :
Melanin 1) defect in PAH → type 1 classic PKU
2) n n dihydvobiopterih reductase → type 2+3
Other metabolites 311 , a dihydro biopterin biosynthesis → " 4+5
-
Alt catabolites are excreted .
Degradation pathway: Aminotransferase
TREATMENT :
Phe .
Hydroxylase
diet Hin Phe prevents mental retardation
Phenylalanine Tyrosine para
.
-
Hydroxy phenyl
-
M . isomerase
PHPP
pyruvate
.
dioxygenase
Alkaptoneuria
↳ causes :
defective homogentisic acid oxidase
dioxygenase PAH Tyr aminotransferase 4- HPPA Homogentisalé
y.malylacefoaceta-e.tt
.
4- fumerylaceto dioxygenase
-
, , dioxygenase
Tyr 4-
, -
acetate
Homogentisaie Phe hydroxy -
HOMO -
Money 1-
g) ketogenic
F. acetoacetase
phenyipyruvicacidgentis.ie acetic
" " to
acid
Fumerate Acetoacetate * Both Phebp Tyr have the same acid
breakdown after 1st step -
oxaloacetate
"
"
Étaie
→ accumulation of homogentisic acid does
acet " ' " A
stimulates
not cause issues other
gluconeogenesis many than urine
air
turning black on exposure to
Rate limiting step
.
→ No life threatening issue
PAH
Phe Tyr → ocnronosis Arthritis
Tetra hydro Dihydrobiopterin
BHY BHZ
-
biopterin ( co -
factor for ↳ bluish black discoloration of tissues .
V
( co fact -
PAH)
, NADP NADPH
Metabolism of phenylanine and tyrosine
:
1) Go over learning objectives
.
.
2) Go through slides
-
.
3) klatch a youtube
-
video .
The rate determines if the
limiting step pathway will
4) Read through book
.
5) prepare notes .
and study .
continue , the amount of product that will be formed
etc
.
this if there is an absence of either
•
Discuss the metabolism of Phenylalanine & In case ,
Tyrosine and its related disorders of the highlighted →
Phenylketonuria
As Phy cannot be converted to Tyr ,
instead it
phenyl pyruvate
which in turn becomes
is made into
Phenylalanine PhenylLactate Phenyl acetate Phenylethylamine Phenyl acetyl
glutamine
-
[All are ketogenic]
•
proteins
proteins
make
are made
up most
of
of
amino acids -7
our body → Phenylketonuria [pku]
SIGNS 1- SYMPTOMS
20 amino acids found in the body
{
'
d e l
1 progressive)
including s
'
phenylamine . m m p
.
It is an essential amino acid i. e it
delayed musty se " " " "
to be taken
smell tighter
needs in diet .
development skin
•
Found in amino acid pools in our body eczema Psychiatric disorders
that exist bec proteins don't have a
mental retardation
storage form .
* No symptoms may appear in 1st few months .
Importance precursor for aromatic compounds
Diagnosis
•
:
required for bodily functions .
1) Check plasma phe Levels l may not be
Tyrosine
-
detectable 3-4 days postpartum ] .ie birth
2) An older + less reliable screening test
.
It is a non essential -
aa formed from Phe Uses Felts to detect phenyl pyruvate
and is an intermediate in it 's normal metabolism in Urine .
related to
genetics
→ →
+ degradation pathway .
probes In
* DNA prenatal "
facilitate
of PAH /
diagnosis defects in
Normal Metabolism dihydro biopterin reductase .
catecholamines TYPES :
PAH
[ dopamine epinephrine
Phe
,
Tlff
,
norepinephrine -13 Tu]
, ,
•
Hyper phenylalaninemias :
Melanin 1) defect in PAH → type 1 classic PKU
2) n n dihydvobiopterih reductase → type 2+3
Other metabolites 311 , a dihydro biopterin biosynthesis → " 4+5
-
Alt catabolites are excreted .
Degradation pathway: Aminotransferase
TREATMENT :
Phe .
Hydroxylase
diet Hin Phe prevents mental retardation
Phenylalanine Tyrosine para
.
-
Hydroxy phenyl
-
M . isomerase
PHPP
pyruvate
.
dioxygenase
Alkaptoneuria
↳ causes :
defective homogentisic acid oxidase
dioxygenase PAH Tyr aminotransferase 4- HPPA Homogentisalé
y.malylacefoaceta-e.tt
.
4- fumerylaceto dioxygenase
-
, , dioxygenase
Tyr 4-
, -
acetate
Homogentisaie Phe hydroxy -
HOMO -
Money 1-
g) ketogenic
F. acetoacetase
phenyipyruvicacidgentis.ie acetic
" " to
acid
Fumerate Acetoacetate * Both Phebp Tyr have the same acid
breakdown after 1st step -
oxaloacetate
"
"
Étaie
→ accumulation of homogentisic acid does
acet " ' " A
stimulates
not cause issues other
gluconeogenesis many than urine
air
turning black on exposure to
Rate limiting step
.
→ No life threatening issue
PAH
Phe Tyr → ocnronosis Arthritis
Tetra hydro Dihydrobiopterin
BHY BHZ
-
biopterin ( co -
factor for ↳ bluish black discoloration of tissues .
V
( co fact -
PAH)
, NADP NADPH
