ellon

osanabol?c font by than|&ssenttal Trcoasketoton

Ano
RSseqtal aent acds)
amno lõund
ataboliom
alanîne ol
CapbonaHan
Ok
nate
Ihe
Gtutamate t--amino Geneoa ÜnGO Y
Amino the An
concantaolfon the Paotelnsare
in (bio
amino acduut
aeîda.
acds
GOcHon Qcirtoaellulasamfno
d
ominoaclod roast
hohfle
psotens,
the loctally and
0cve
uboth andu aclods mechantasm
non-Oxidotr
nmetabolsm toanspogh &nteo ac?ds ha Usea
o fmpontoni
favolvac tamine pool abou ntsocen-Contalnfng
men-malecules
halconising
< a
nouas întaacetlular
the okout Cyele.andamine q o
t0 the
haehavebe
Cells "toethes q 00g an
oepeattnd amino
QmfaoAclol
deamqaten
n fe aclds. 10 the to be Ominea)
aicdos
ale a okne cunthastzed b
and acainst amino bhdy.
o arpgltomnce. Hs actal
Um
milatfon
cuis. ConstBute
he amlnD
paodled untks. ond ,
glycagenicDeamlaatfon
melabolkum
acds oareaaínaton,decantoy
deulphunatfan.
atabollcanad concentaton
a body by O DATE PAGE
Rs Obouth ac?ds n No.

aboub the hethe agyntheatu and

alnoy pool. ao
oldor
paotetngand ihfch dei body do ketogenfe
50%,Qnd omiho Cahabi
and hohe CeR~onid Cnon
g gapn actt ond
 becan 8otha
?t molecule. soluble ateo land
o beConvosted to ammon?o
ha thRY Thin<onQ
blood, thmolecul2S
o
amd eaoton th natuxe intooxe becomes blooo
Then blood th9 incgeaeL
th blood,S0
psm +
mp
Omino som iommon?a
got 2paoduct
stod0lucosa
at. ox £ne~sy roovide to Zo
Oxo ads olanno fntake &rcass tã
hydaotagistycogeH)and
em lipds tos as
iGtoag no sTheae
METABOLTSM Ac1D AmanO OERN*EHL
OF An
acfds amino
tfa&ssen on- FAT Gtacou
Ac+DS Kero
UREA
NON
Gutomate
(IRAMGAMINATTOnN
utaoto ketog
ompoun 7, - SyhegRS
nthaSta AADs AmTNO Proten
Paoteln
Grooteen
Gody Dfetary
 ( PAGE No

ts
in the
Sunthestzed b thethe sea
\OATE

ycle hich occUSS

ne heenafhfng
in th
fost o< amino actd
oterseoernova) o
kOm ot keto actcds.
Thesekofo aclds ohs
nmd tals post Yntogsated
paxt in the nengu ofth the kaeb.
meta bolfsm,
URlfzalon o amino
Qmino Qctds
MASt o he body actds DM body (00)
th amino antetrts desnaded ae Sunhastad fson
cd Qool, Thase Pncud Enzyme, hosmones, iru
nogsotefns contsacRlo graotoBns
may fmpostant nttsogenous
Pygimhtlnes &t) e podicdcompound. a amino (Quntia
som thi Cpoxphyans.
atda
Genesal Qbout O-154 4 body enesgy gequiamants a
met 0n thu amfnoi0ce
Thu omino cdg ane corxottod to
ats,
TRS bocomes poedomfnogt ohen he pmotetn conGumptoz
casbohsdotegiand
fs
the body gegusernenhs,
GENERAL AsP£CGS OA Amino leips. MESA8OLTSM
4 Th anfno ac?ds undengo cestain Comman Racttons is
gonsnmfnatfon follasNd by leamiegton (osthu lboxaton.
o ammonla
the ammono acRdS Rs uIf2d ox th
Osmatfon oUgea hch Rs an erretoss end pduQ
poton metabolRgm, ConYeT
Ihh Castbon skaletono
tod to koo adds tocnSamfoaRon) hcn
moxo ol th Cby oates
9ORIzed to
glucase
 amino acios
acfoks PAGE N0
2cept lin,
yht, haoonfne , roltne
panHclpate fa tganhminatfon
DATE

Mechanfsm 04 ARansomínalon
Qnd

Sgansomi
oner
phosphate to
0 othe
atfan oceun& to trio stages
amino ndup to
anino Ronm mdovanfrne qhosphaU
the
tonaama ido xal 4

actd ond hen

Osfdoxârntna
Hxanstased to a keto act
faoduce phosphae fg thtn
raume
Hs CH CO0 Gutamote
ysuvae traniam¡u
CH3-CC00
dlanin
Camtne acto
(hoacd)

Hoidoxd hosphae Pysscoxamine phaspha.

CH H2
Gutam ate OyUYo,
transamHa!
Gutamate angwara
Ckeioocd)
Cam?no gcll)
dlani
tosmaHon afglat (Pyuvate (Ketoacta)
Camlno acld) bytoaaminalan
 ammonfo

cooH acetate ofesee

CH3 aipestate.
om
OxaloaceoBo
CH ktdney
Rsto
CO af Re
Rbeatfon Iberantfon
ó andnmtatan
Qcidks kalo
acfds
ketaglutaxale
Q- Skolton
PLP SGOT ros
Omîno
the the
dooH Qutasatt Synthes
and
Q-
CO0
asbon s Qf în Dxfdattn
DEAMInlA19ONN
Ctl2 -keo ToSutts actde. mosts
COO CH goLup
cleaminatfon
H3
RS
deamenatfon,
the
DEAMnATIOn keto
(Gfmutt³ncouuslu
oxfdattro
lon takes
place
om o
amno
the
Ro
foamatBon DoamBnãlfon
puspose
CO0 CH3Alanne Aspesdate to
,n-c-H
COOH OxfdatiYe
Coo
Convegted
Eis The
* 4
 praodue
dutome
cotoyudJA. throygth amfno
Coo CH2door dehydaoganau
?besxate by
ammon
Shgx hydsoxy
atatycsed
ollora fnínodutorote
dearângiton, oceuss ohydrataw,
utoi
u2e
NO. Rbesare deminatec
PAGE
N
DATE aho
to tasahe Q-Iminoclutazate the deamioatfon,
deentne
CO0
a-ko
can gutamaa Cdehydnatac)
homodsexfne.ase
the to ofdatre
to Rt_ gluu-
(GDH) keto
tanSarinalfon,
genenatßon, ENH
Oxfdaon.
undesaolng Q-Tmfno
toansoedthat Intesmadlote be homoexf CH3 acfo
too
NADHHH
undesçot u- Coo
DEAMONATNonN
mn non-oxfdatve
to
dehidsnenags by
9J
CO
Enzsme.
glutarate glutamatt acdscindexoBng olehåcdsa
Enegc se
and
apidly an GOH amno J
Jtau
O addG thaeon?ne
poocas8
hclucing af o
NADt
L-GutamoteOxiDAINE
NOn- deperndent
eundexgo
aalutamatefogmaHon ge the bothout
Gutomate Coveaoson
amino
Cidattan
Co0 +Bonin,
hth
nctton, COO CHn TayPIP- Coo CH2 0Serl
acRds.
nst nHa
th
,
 butyo auo.ghenylalanfnalcisot Enzumephenyla CH-CH
(sknkCCns)onlox&ntphoín
Melan'h
gan Enym Dapomine
odshydxatl
n désosdels. 'metabolkraessire epinephoin
coudby -
ThTeon?e
coOH hydxytau
METABI
fh heratfe
thoeon?ne.
H20
acto
U-Tm?no meaboe
celects Common anQutosomal
hogma
posson,
hydaoyt
metabolksm H20
CHn AC+o the phenylaonhe Phnyt
ao
&yme the
moast
9 alaniu
THmshe
o Amgno tto
adeaminaton Leading datcioncg to
dehydratau o akvesal metabolfsm,
by
disoadex CPku)-fs ud conventolsm
in
ASMetabo
H30 CHco0
CH
gena.dachodaton he ohA
OF

Oxfolaie PLp
DisoRDER
Inbosn
deteny
to hydrsokyau
snFot Phan
QRCeSs0Ye PasnULKEONURIA
nuto
Phenylketo
ac?dduu Prengtalanine
phonylalanfn
-
nlon
Coo
H
InBORN y0sin
tyaostn1 fs
amtho Du
to
SOmal PKU
lanln
* in
 nd toanpot
&
phenylaia bapopho
ncsoud
heoh ths
PAGE
NO amumualBon fnpatas
actolu
DATE
metabolism n
ig
(keton).
pysuvae
Phny
n unfne henyllactae of
oeAu onsomfnaw ytem:
manglastaloni to
baat,amêno
aouttnephengtactylgtam
In
potmanty
cause
Pkuth
tO_
QMtinate
pathrayvcsetod dus n .matte
Qvom
Ond Phenglaianf
ngn Quo
CCuns
n blood,
aphenslal
Rn hlood the
RSSueland fhenylacetaQuantolbiochumcal
Contoo
wn
neovou talk OHs
metabolfsm 'n ui Qhenylacetyl
MAbtNADH+Hta
OH
`ymploms
anol
dstunbancOmeBabolftea aotoncotfon
>
menta
Ond oMumul
,
Phenglketonuafa Chenalaehte
voalk metab
Ohenylalaning tactobe
lactae.
cvetod
hGil
Symptamjects 2farluse..to
Ihs
to in
Pku
alonfh Du phenyl
Qnd
Rs
 neurottOnSmittey) is cOnt dlet
typophan timou TRs
nendbogn phenulalanin
PAGE
NO.
Competttrely femfc by Concentaafon the
the csynthezodin
-?n
DATE
fomaon,Thi phenalatanfne don
to Dm bu
Skin
colout beooride
due nofth the
detected
be
vcfhaBomy SyntheNfzed ooeenlngcan aluo lon
nåaJanðng
metabotftu hendlaJanfn ootecte
bo
noth
aseaotonin o7 be Can be
Canfnsuetent melanth teat
lerels
deBectodby
PRv ph
{onos cshowd
Cankot
Q8oment Guthae
aenotonfh
amdis Eect
on
plámentaon that Oe
platma oplama alectfngamd
ojo o fmpafas UVOH
tiatn
Yn o Cultubod
amnloc
ceA. tycaosrs
(Syntheste gderumulaiton
that of
Pku
Diognosts
poplamantaifon diognostsIaeatment
o,
PKU
the mostu
bs
th
noamo)
Tonge Sutctint
9uanity
Esenta)
ol phenglaanin fneneaud
out by
mántenaHe
afynthoufs
SOm
tuptuphan fs Amd doneadd
sVth) yMelontn fs
cosfod +Rrenain)
Phenaly
ynoshau
astna becomeA
Rs Amtno
PKU thu
mpata Thu TRla
Th kor Buu
A * * + {t