Camlin Page

Date

Needs vit B (PLP -

Pyridoxalphosphote)
as cofactor (60/)

o-L6bord

Glucose
Gluccse-1-Phosphote
Gtucose
-PO4
AcHon of
Gly Ccaen-phosphorylase actvity continues Giycegen phosphony
HIL 4 residues are left beyond by 1,6 bondir lase
irisaccharide
To deal with remain ing 4 residues, s20orons ferose.
isaccharide tronsferose comes &Cuts trisaccharide Debranching
binds it to d-4 bond aclvty

After the trisacchoride is remoyecl, the last residue remolns

exposed We cut his d-y6 bond using DseRANGHIN
gCTIVITY

Glucose- 1-PO4 Cytosol

Phosphcglucomuase
Gtucose-6-POq
S r e i haiqscgsve14
P Gttucose-6phesphetese-
Gilucose
GLUT-2
BLOOP
Glucose
 Camlin Page
Date

MuscLE

*Muscle lacks Glucose-6-Phosphatase engyme: in

endoplosmlc retculum.

Glycogen Skeletal
musele

Glu-6-PO GIycolysis
benovo u tili2aton

AFP APP
Glycngen Gtycogen
Phosphorylase Pnosphonylase onioisnPhosphorylase
Kinase

IN ACTIVE ACTIVE
Adenylyl CÁMP dpndnt
ATP cAM? proteln Kinase
Oyclase
Phosphorytase
cinose (DP

In
case of Hypogly.cemio, Gilucose is recquired whlch is
vSianalled by Glucocon

Why Glucose-G- PO in skeletal muscle2

Bes during ExerciseStvess Condilion)

Epine phrine & Norepinephrine Stimulates

CAM
Gqlu- 6-POh for enerqy in muscle for oNereom
Stvess
 Camlin Page
Date
Recephov
Glucagon Livey

waEpinephvine Liver
Novepinephrinecisdionn Skeletol muscle
Galucoggn Siorage Dsorders
ype Gtlycogen Storage Dicorder

Deficiency of Glycogen Synthase

Hypoglycemic episodes
Ketoacidosis

TypeGycogen Storage Disordes

Ja Von-Gierke Ds
Type

Von- Gierke's disease La 9ovigoaui

-Glucose-G-Pbosphotase deficiency (Dn liver)

Glycocen boc
anolsy
LacHCAcid prdcth
NGlu- 6-
Phosphatãceiycolysts
N o Glucose Ino blooc

Boby will have Hypaglycemia

Free fatty a in blood
ketone body in blood -Acidosis.
Lachic acid.
AUric acid.
 Je
Date

b
is normal but having samne
Phosphatose
Glucose -
6-
manifesta ttong. g s g e v o

LIVER

Cochic a
tiGlucose-G-Poy
Glttese--6-Pe
ER
ots Aranslocase
(GLUT-

x No Glucose

GLUT-1 is mutated. Glucose4 cannot reach lumen

1rarsportey is deficient

otypoglycemia
viti leA Lactic acid o
uric acid
oFFA in bood 9pvia
o Ketone body in blood

esihcgaco9-3