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9393033904

CARBOHYDRATE METABOLISM :

> Gh£wÑn→% GLUT

Portal View
Egeption

gestion
Fructose
Intestine
Galactose
maltase
Maltose , Glu + Glu
sucrose sucrase> Glut fructose

I Lactose Glut galactose


lactase
>

Carbs
i. The end
product of digestion of .
is

Glu Fructose Galactose


In Liver converted into
Glu is
Glycogen

Glycogenesis
-
-

-

quit Glycogen
t -
t
s -

brlycogendysis
↳ twose
Tanter
-
I

Nat
Nat InDependent Dependent
GLUT SGLT
Name Location Features

GLUT-1 Brain Basal


uptake of
-
=>

GLUT-3 RBC's
->

Glucose
[Ability to attract
- Placenta

glucose
even

cusing Hypoglycemia]

GLUT-2
-

Lines ->
It allows both

kidney enticy
as wellas
-

of geueose
B-cell release
of pancreas
-

GLUT-Y ·

Skeletal muscles -> Insulin


dependent
Adipocytes transfsostes
-

-
Heart

**
GbUT-5 -

speematoxoa
->-

Transports Fructose

-Testes
-

Intestine
GLUT-1 Endoplasmic Reticulum
-

(Line)

Nat Dependent
SGLT-1 Intestine
Absorption of
-
-

glucose .

SGLT-2
kidney , peabsorption
·

->

(PCT cells
of glucose
.
19th July
'
21

Oxidation :

1- Oz
-

Hz
e-
-

Bedim oxidation number


feta
:p Fey feta
of "
Odidat
feta > fet3

oxidatn
a) I
-1
, I
Iodide ion Iodine
'

Substrate aid"
> Product

Hz -

"TdALYtoTu) : NADT
FAD >
-
NADH

FADHz
+ H+

ETC Electron
Transport Chain)

AYP
-
ETC ELECTRON
Aka
TRANSPORT CHAIN

Chain
Respiratory

location Inner membrane



:
of Mitochondria

protein complexes complex I


5 NAD
:
Dehydrogenase
• -

complex I
-
Surinate
Dehydrogenase
"
II ·

Cytochrome Reductase
11
Ñ -Cytochrome
Oxidase (co cal,
,
ns)

11 ✓ · -F
,
Particle


Mechanism
of ETC
g-F-F.mn#--

!
• Matrix
" ""

¥++Hµ*II¥
-

NADH At
- - -
-

+
-

<
-

"

*
-

+
NAD
-
i

EIHXTH-j.es#ET+Eotnoiside.--FADH2
-

Oxidatn ?

-
-
-
-
-
-

]
-

l !
I -

④I•¥
-

→ FAD
-

-
- -

I 02
I
Oxidat
"
II •
→ Hao

+
mil
Phosphorylate I%=yADPtPi ATPsynthase
> fft

It.ro/#.-iI OxidativePhophoylat "

In ETC Oxidative
Phosphorylation
°

ATP
• .
,
is
synthesized by .

1 NADA 1- Ht > 2.5 ATP 's

IFAD Ha > 1.5 ATP's

synthesis of ATP 2

Oxidative
Phosphoryl Substrate level
Phosphorylation
"
at
NADA + Ht FADHZ

Complex - I
v v

coqfvsiqu.mn Compl e x II -

carrier
NADH
dehydrogenase Succinate dehydrogenase
Compt e s Ñ
NADT
"
9-0 1^-0 SFAD
Phenobarbital -
Matonak
Rotenone Cytoaome Otaki Antimyein ,
BAL

Ghc
-

Oz
Ñ
Complex
-0 oxidation
cut,8⑧ > -

Iydase

Has HÑ
cytochrome

ADP t Pi

Oligonyein
-0
>
Complex Ñ '
-
Phosphorylation
> ATP
Fo -

F
particle

Mobile carriers
of ETC

1.
Coq ubiquinone : bet
"

complex I. I & II.

Cyt bet
Complex II & I
"
a. -
C :

Nncouphrs
1
:

It uncoupled onidat " &


phosphorylation reat
"

: 2,4 DNP
egg
Aspirin

Physio3logical Nncouphrs :

i. Thermo
genin
(Brown Fat)
a.
Thyroxi
Free
ne acids
3.
fatty
9. Bilirubin
Act
Glu
qLUT
% 021GW @ c) X
am
Glu
02GW
1 Aerobic
1
'
,
Glycolysis jibnaaobie
cytosol ighuotyn
's
1
t
v

Lactate
Zpyruvate @ c) - ✗ →-

Inform Intermediate
'

step Pyruvate @ c)
Mito
Mitochondrial
Matrix
sink
reaction { Acetyl
vPDH(pyruvate dehydrogenase)
CoA @c)
*A

Oxaloacetate # Citrate
QQ 164
KREB 's Cycle I
f
i 1
Succinyl GAS A-
ketoglu@tarate
@ c) c)
GLYCOLYSIS :akI Embalm
camp) Mayuhoff Pathway

Glu >
Pyruvate '
"

Glucose
°

Cytosol .
- c- c- c- c- c- C
-

kinase : transfer of Pgp .

from
molecule to other
µyp one

☒< Glucokinase
Hexokinase
.


Reactions : ^

Glu -
b -

p
c- C -

c -
c -
C -

C -

Isomerase
ttsomuigatn : same

structure
format
diff
F¥_ 6 p
a
- -


- -
-

AÉP Glu 661+1206


• -

c-c- c- c- c- c -

p
o
few ↳ HROG
ADDv☒ 1)
-

PFK -1 ( phosphofsuktokinase -

cruel
mutase : ⑧
P -
fuel ,6 ,
C -
C -
C -
bisphosphate
C -
C -
C -
p
-

moving
from one 'C b- another
'

'
C' on same molecule

v Aldolase v

DHAP G-3- P
dihydroxy*
acetone
phosphate NAD+
Pi
Glyceraldehyde *
-

Phosphate
3-
G- 3 -
p

dehydrogenase
>

,
.5atp

bisphosphoglyarate
.

a
NADH + H"
1,3
G- 3- p
ADP
Phosphoglyaro
? "ACI% ✓
Kinase

Phosphoglycerate
µ
3-

MUTASE

2-
Phosphoglycerate
H2O # ENOLASE
(PEP) Phosphoenolpyruvate
@P)
ADP
pair Pyruvate kinase
ATP ' ✓

pyruvate

Energetics :

'
Mechanism .ofATP
Enzyme No

NADH -11ft b- ATP 's


1. G- 3PDH 2 ✗ 2. b- ATP 's

SLP
2.
Phoqphoglyaro
kinase
LATP 's

Pyruvate /kinase
3. SLP ZATP 's

4.HR/Gk -

IATP

5. PFK I
-
-
IATP

Total RATP

Aerobic 7ATP
i. Net Gain of ATP in
Glycolysis is is

Arcusobic n 2 ATP

10T¥
-

Irreversible Reaction B,
t.HR/Gk [Ist Step ]

2. PFK g-

[RLE]

3. PK [Last
Step ]

Regulation of Glycolysis : -

Rate limiting Enzyme @ LE) : PFK -1

Inhibitor : Fluoride
-0
> Enolase
(NaFS

HK vs GK Glu

all tissues oliver

✗ • Insulin v

G- G- p

① Aerobic
Glycolysis
Anaerobic
Glycolysis
RBC


I Oa 0.x
& &
No mitochondria
mitochondria Mitochondria
1
Anaerobic
Glycolysis
Mitochondria
8

=>
RBC has Or but no

So No
Aesobis Glycolysis
.
Anaerobic
Glycolysis : no 02 & mitochondria

no

Glu
Poconori
:
ATP

G- 6- p Eg

Five -
G p
-

ATP *
Fsu -1,6
bisphosphate
L
>
DHAP
NADIA
G- 3- P a

3- P

i
G-
*

NAD V '
NADH + H+
1,3
bispg
}
v

NABHT 1,3 ATP '

bispg v

ATP 3bar
{
>
.
1
3.
by
I
2Ñg
{
I
2Ñg

b
PEP
v v

NADH + Ht
NADT
ATP
PEP < ✓ a

SATP
Pyruvate > LACTATE
Pipuvate ✗☐µ

lactate dehydrogenase)
LATP
:O Net
gain of
ATP in anaerobic
glycolysis is
* Clinical Co relation
- : -

-Y
Anemia
1.
Pyruvate kinase
deficiency : Hemolytic 3 Not Natkt ATPase
No being bodies

- -
-
- -

.
-

2kt

Pyruvate Kinard Pkd ATP &


Nat
-

nay

doe ATÑ
.

synthesis -
-
-
-
-
-
-

"

failure of Natkt ATPase


accumulate of Nat Hao +

ftp.MOLYSIS ANEMIA :

LINK REACTION
3
conversion
of Pyruvate to
Acetyl co A. -

Cumin
Oxidative

NAD+
Pyruvate ② c) thymine
to
PDH complex me -

enzymes
5 i.
Lipoic Acid

H"cAt§
NADH + Pysvale Dehydrogenase 2.
it )
Tppftlhiaminepyrophate
to A
-

④ 3. CoA

4. NADT
so FAD
Location -

Mitochondrial
Matrix

KREB 'S CYCLE CITRIC ACID CYCLE TCA Cycle


Tricarbonylic acid
cycle

Acetyl 6- A @ c)
-1 citrate
synthase
OAA
^ @c)
> Citrate @c)
NIDH NADH + Ht
>
Aconitase
(reversible) µ, , I
* µap+ ✓

Malate Cis Aconitate


-

fumarase µ µzo→✓
Aconitase

Fumarate Isocitrate
farm.

[
>

SDH
*
FAD NAD *

Succinate NADH + He
I④ÑRLE
ADP
[ succinate
thiokinase
NADH +
a
H+
,

·
stiff succinyl 6A
NAÑ *
to succinate

④)
,

2- KGDH
L -

RFT
(S2)

I. Net
gain of
ATP in KREB 's
Cycle is IOATP
from each
molecule of Acetyl G- A
Krebs
Cycle : -


XKGDH : oxidative
decarboxylation
• 5-
Coenzymes :< VitBcompkxwM Vitamins Krebs
liloic acid reqd.in
TPP > Vit / thiamine
B, cycles .

CoA > Vit Bis / Pantothenie Acid


NADT >
VitBz/ Niacin/Nicotinic Acid
FAD >
VitBa/Riboflavin .

Fructose Metabolism : -

nosymptoms
honey >

① y
-

fruit juices Essential FRUCTOSE TT


I ✗
fuectoseuria
kinase
fructose

Jerome {
V
② Fructose -
I -
p

Fructosemn.TL
V
intolerance aldolase BX
qµ pmg.se -

sorption
DHAP
Glyceraldehyde
j V

Metabolism
Galactose Metabolism :

Mike
Galactose
-

- Milk
products
Galactokinase

| God !g

{ { Yum
.
-
-
-

p D
%dÑd
-
.

UDP Glu

'

£t"
-

" " "


.

"
" "
" "

Transferase (GALPUT)
Galactosemia
Galactose Glee
!1 -
p
Glee
-

Galactose

Metabolism

" "

Oil drop cataract

-
GLUCONEOGENESIS :
~
Protein

def : fromaticmAminoof Glu from


m&×apt
non -

carbohydrate sources .

egg
Acids
Acid
leucine ,
lupine
IPG -
A Pyeuvic
Lactic Acid

Glycerol

} cytosol Mitochondria
tocat " : Liver 90% +
-
.

kidney
10%

? will not
☒ glucose -6

phosphatase
>
Glu Dte : Gluconeogenesis
I occur in Skeletal muscle
G- 6- P bcgofabsenaofG.lu G- -

cytosol Fib phosphatase


friar
¥&spn%%
P
enzyme
> -

1,6
v4
f- 1,6
7
bisp .

RLE
DHAP 3- P
G-

iiosbpg

Zpg
V

2pg
V
PEP
MD "
Malate > OAA cartoony kinase > pep

Ñm€,
Matata
Mito MDH
OAA @ c)
gy.carboxylase
ua#Pyuwate&d
I

Biotinlaswengyme)

CORI 's
Cycle Exercising
Skeletal Msl .

* one ,
%
>

!
Pyruvate Alanine
+

amimpymate
tractate

(lactate

Alami?
- ← LACTATE

< Alanine ←←

/
Alanine
<

Li
Blood Vessels

Gbu -
Alanine
Cycle Cahill Cycle 's
GLYCOGEN METABOLISM :

Structure

of Glycogen :
1.) Storage form of glucose .

a) Glycogen is polymer of glu .

3) " "
a branched polymer
G-
Go

:¥÷¥÷
-

:-c
,
-

a ,
^

✗ It a) Glycosidic
> bond At the BRANCHING POINT
}
✗ ( 6) Gtywsitic
→ bond
.

a) Glycogen stored in Liver and Skeletal med .

&
Comore )

GLYCOGENESIS :

def :
Glu >
Glycogen

location : liver
skeletal mud . Cytosol
• reactions :
G
I

G
Protein
5* branching
**
RLE
4
Gcogenin A
enzyme
>
GLYCOGEN

@I}
synthase
I -

D
>
linear chain

UDP -

Glu


activation

GLYCOGENOLYSIS :


def : Glycogen > Glu
• location : Liver & Steel .

mslifcytosd .


" a-

React a-
g- semoved

I
g.
glucose
G- G-( (G. G. - -

G. -
-

G
-

G -

q -

q
-

for &
-

from end ↳ are

A left before the


.

Glycogen
• • •

glypgiogporyhhs glucose -1ps glycogen phosphorylase


}
brauching point

RLFC)
V
G
G- G
-

g-
Ge G -
G G -
-

H -
G -

G G G
-
-

3
Limit dextrin

debauching enzyme
V

G -

G -

G -

G -

G -

G -

G
linear chain .

Glycogen Phosphorylase
v

Gbu - 1- p

Mutase

Gbu - 6 p -

Glu -
6- phosphatase
Glu
Glycogen glycogen phosphorylase✗ VI
Heros
branching >

cytosol enzyme
linear chain } limit dextrin cytosol
glucagon synthase
> glycogen
¥2s:)
Tiffani .su
debranchingengyme
/pomp Aajidualtase
"
UDP -

gln Ti linear chain


type dis
akoilysosomat
- .

Gop
activation ,F u
glucosidase
Glu Glu -
l -
P
1 Mutase
dysosome Glu b p -
-

1 / Glut b- phosphatase
dis
Glycogen storage :
Glu
Name
Enzyme deficiency
1.
Type -
I :Von Gierksdis :
Glu -
6-
phosphatase
2.
Type :
-
cows dis :
dekranching enzyme
&
.

Type VI : Her hepatic glycogen phosphorylase


M

:p ?s f)
3.
-
:
->
-

⑦ Glycogen
.
Glycogen
muscle
Gap
Gop
.

v ~

I Yoon
HER 's
/vI Mcttrhde 's
I
I Pump
2 A HI Cori
A- B C D
II And
Cori's/
If
Anderson / I
{ I Maker8
's
...

branching debranching VI her

tired
Type I Anderson 's dis
a. :
branching :

enzymes
- ->

Type I : Mc Arkle dis muscle


.
-
-
's
glycogen phosphorylase
:
=>

Type II : Pompe dis Acid maltase Kysosomal Guosidase


G. 's :
Hmp SHUNT RP
hexose
:

monophosphate Pentose Phosphate


.

Pathway -
-


Jump Pathway
.
2

M Fatty acid Sym Lipids Syn Cholesterol Segno , ,


Steroid hormones

-4 RBC (Removal offree Radical) (40c]


,

sign
1) NADPH .

Neutrophils (Killing Bacteria)


d
Ribose b- P DNA & RNA
2)
- -
=
.


Location : Adrenal Cortex
Testes
Ovaries
Placenta

Adipocytes →
Cytosol
Lactating Mammary Gland
RBC
Neutrophils
Eyes Class)
Reactions :
Glu b P
"

- -

I NAD.pt )
phosphate dehydrogenase
*

① idat "
p☐§w .co

1 g. g.
>
NADPH + Ht

y
t
Fru G p
-
-

Role of NADPH in RBC : Removal
of free radical Halk .

µµµµ!¥h¥hi
g÷¥µ②,µ °
Glu -
b - p
*
I NAD.pt
}
Oxidat
I GGPD
"

leads into
, camping
t , • • • •

Fru 6- P -
•• • •

being bodies

1. Glutathione Reductase ¥
2.
Glutathione Peroxidase
µ Co enzyme) -

Anemia
w GGPD deficiency leads b-
Hemolyticbodies
-
.

Heinz +
-

Anemia
-
1st mcc
of Hemolytic

Role of NADPH Neutrophils :-O ,


dependent killing
is
of

bacteria .

HMP G- 6- p
shunt
& I NADPT
I GGPD
I NADPH + Ht
neutrophil > 1- Oz

4- 5 lobes ) d
F-Gp ]
Hyon

Kill bacteria

GLYCOLYSIS HMP shunt

cytosol
ATP
cytosol

✗ CO2
Proteins
Chemistry of : -

Proteins Acids
:
polymers of L d-Amino
-

okay
polypeptides lA¥ÉA? -
AA -
AA -
AA -

AA

peptide bond

Amino Acids : -

• It is a carbon
compound
acid
e- consist
of Amino
group -1MHz)
&
carboxylic group
. -400M)

,R(Alkyl group)
Ntfz f COOH
-
-

*
*
R
o I
C
Nltzt
cool
-
-

H
tnt in our
body
z
+ relation
Sons Twitter ion
-
ve Anion
**
classified of Amino Acids
"
:

I. Based on structure -

Tgp
Aliphatic AA Basic A. A
&

1. : 5.

simplest -

Glycine lysine
Alanine
Valine
arginine
histidine → Some essential
PB.LA
"{ Leucine
Isoleucine 6 .
Aromatic Ant .

2.
Hydroxyl Gp containing
.
A. A.
Tyrosine
Tryptophan
Serine
Threonine
Phenylalanine
7. Amino Acid
5- AA
3.
ulphur containing Proline
Cysteine
Methionine

a. Acidic AA

Aspartic acid

Glutamic acid

Asparagine
Glutamine
II. Classification based on
Nutrionalreqd .

-
Essential A. A (not produced in
body)
eg.TV till 8pm .

Non essential A. A (
produced in
body)
-
-

Rest all
og
.

Semi essential A. d-
-

(reqd.in growing children ,


QQ lactation
,
Histidine
eg
.

Arginine •

III. Based on Metabolic fate ¥

A. A
1.
Ketogenic
eg
.Leucine > Lysine
2.
Glucogenic &
ketogenic
eg
.

Tyrosine
PITT Tryptophan
phenylalanine
Isoleucine

3.
Ghecogenic AA .

all
eg. Rest
Codon-
Stop
o 21st AA :
Sdenocystine UAA

UAG
I
UGA

-
-

formed
codon :
by
UGA
SERINE

22nd A. A
• :
Pyrro3 lysine
codon : UAG

PROTEIN METABOLISM f.
Protein Rich diet

deaminat
"
:*
!
D
.

go Proteins

r v AYA
Ntlz Keto Acid
Ammonia

7÷: ↳ UREA > excretion ↳


UREA CYCLE ORNITHINE CYCLE KRF-BHHENSELF.it / cycle
location : Liver cytosol + milo .

!e÷÷i÷÷÷**
Ammonium Jon
Nflgt + coz
Carbonyl Phosphate Synthase I
-

cytosol
Ornithine otolornithinetrcanscarbamoylase

3
NREA arginase
,
arginosucciinate
NH , CO-
-

Ntlz b- synthase
imginosuccinase ✓

Arginine ✓
A

Fumarate
Arginosuccinak

• Source
ofwua : NH , - CO -

Ntlz R
I

itxpartak
"

Ammonium Io .
->
At NHst- C-COO-
I
ion
H
Metabolism of Aromatic A. A
Que
Tryptophan

TyÑsine ①Melatonin

Tryptophan ② Serotonin
/5-H5

Phenylalanine ③ it-By/niacin/Mctinic
I
[boing tryptophan -> Longniacin)
peartrop's d/1
42
Absontice oA A From intet
Metabolism
of Phenylalanine &
Tyrosine
.

-
& iryptophan
e Pallagra likerptur
F
phenylalanine
0VÑ 5) Tyrosine
phenylalanine Johnathan
-
OH > Melanin
Tlztq
Catecholamines
( DA NE Epi)
, ,

Idopamine Nosepis
,

PA
CONVERSION
of b-
Tyrosine
P.A. -
# Tyrosine
on

>

tetra
hydro dihydro
biopterin cbiopterin
CBM) (BMeS
Ck :
Phenylketonuria (PKU)
PAH &oft
enzyme deficiency :

CIF : pale skin 3


Ashore hair in Melanin
Mental Retardation -
-
↓ in Ts 4 Ty
Mousy Musty odour

FORMATION of Melanin
tyrosinase
Tyrosine > Melanin

Ck : Albinism
vitiligo
FORMATION of Tz Ta > follicular cells :TzTq

i.IT?i-8.:i.Jii- A- cavity
Para Cells
follicular
¥ %jo
> -

a
. C- Cells
Calcitonin
-
>
z

Sakitoner
sympostudvi.SI
-

Natal


Ñh"d"*

÷÷!÷¥
>
follicular cavity
-
"
I AA-AA-AA-T-AA-A.tt
oooo
Tyrosine
>

1
g- I
> Nt
.
-
. :*

d -•TPO > I
tyrosine tyrosine

I
VV

Tetraiodo
✓ v

Triiodo

thyronine
&

thymine

Thyroperoxidase (B) (Tg)


I
akgtysox.im

Formation of Catecholamines :

P.A.

PAH
-
OH >
Phenyl Alanine Hydroxylase

C -


OH
tyrosine
>

DOPA
TH

(dihydroxy phenylalanine)
Tysosine Hydroxylase
DORA

Cozi

decarboxylase
DOPAMINE

DH
-

OH >
Dopamine Hydroxylase
Nfethyl ✓
Ctb Non
epinephrine
Fmdu%kn%K SAM
-

methyltransferase
8AM a

EPINEPHRINE
S-adenosylhomocysteine

Degradation of Tyrosine :

Pathway Needed
Nos

tyrosine
-

ttomogentiHomogentisale
sate.tt **
*

I
I Oxidase

Mateyacetoacetate

Fuimarylacetoacetate
fumarate Acetoacetate
CK :
Atkapbnuria Black Urine disease e

enzyme def : H 0. .

color
CIF : urine
-

on
long standing turns BLACK Coke .

(
Ochronosis accumulate
of HQ .
in the
cartilage pinna
, ,
sclera etc
,
)
TRYPTOPHAN -

Anp products
. : i. Melatonin
2. Seretonin 5- HT

3. Vit Bz Niacin Nicotinic acid .

Ctc : HART NUP 's Disease


3
A. A. Intestine
• lose absoebtion
of in

o
b TRYPTOPHAN

CIF :
Pellagra
-> like
symptoms .
Metabolism Aliphatic A. A
of


Glycine
Alanine
Valine

SBCA'S

• Leucine
° Isoleucine
*
GLYCINE :

Man : s :
Simplest a. a I smallest
most abundant
s : a. a in
collagen
Protein
3 : bends
flexibility in

functions : >

1. home
synthesis Ltdb)
Succinyl to -
A +
Glycine

s -
ALA

Home
Purine
2.

CaCñN7
Ring formation I


Glycine

i '

¥
3. Glutathione formation
3
(tripeptide) Glutamate Cysteine
- -

Glycine
4. Creatinine

G¥M Glycine Arginine Methionine


-
-

5. Neurotransmitter

6.
Conjugation of Bile Acids
ALANINE
&

Glucose Alanine-

Cycle Cahill 's


cycle .
j

BCA 's : branched chain A. A

Leucine[}
Valine

Isoleucine


Branched chain
✓ keto Acid
dehydrogenase
Products
CBCK D)

co
3g & epyeuvate
complex
Dehydrogenase
enzymes
-

->
Intrate

areti drogmas
3
✓ e

leads into : =-
Lipoic Acid
deficiency

TPP [Thiamine Pyrophosphate)


} Co A
[vit B1) .

Maple Syrup Urine dfs


+
. NAD
FAD
BCKD
enzyme def :

CIF : burnt
sugar
like odour
-

MEGALOBLASTK ANEMIA

Vit Brad or Folic Acid &

9
homocysteine P homocysteine
• •

GAP
methylmalonyl

(MMC)

Metabolism b-
of containing A. A .

Non -

epinephrine
1µF , >
Methionine > SAM -

methyltransferase
homocysteine
/itB1
methyltransferase
Homocysteine
ftp.fthyl - - - -

r
SAME
Tetra
Hydrofolate b- adenosyl EPINEPHRINE
T homocysteine
Folic Acid
VitB9
Propionic Acid Pathway

FÑendmnµf¥→
✓ : Valine
0 Odd chain
:
Fatty Acid
M : Methionine
1 : Isoleucine
Propionyl to A

T : Threonine
coz , Propionyl CoA ¥

,
carboxylase/Biotin
Methylmalonyl CoA (MMC)

Methylmalonyl CoA
mutase
/Vit Bit

Succinyl 6A

Metabolism of Basic A. A
☐ Histidine
*
decarbonylation
> Histamine

Arginine >
Urea Cycle
Nitric Oxide (No)¥
#
L
Arginine
nitric oxide synthase @
% No
Topic : LIPIDS

chemistry of Lipids /

lipid : Esters
of of fatty acid and
glycerol
organic chemistry :
Estrification
carboxylic acid
COOH
+ Alcohol
OH
Ester t Hao
É
-
-

3 I o
-
- -

fatty acid +
glycerol → Estee Cupid)

§ -
FA

Y
-
FA
e
F-
-

FA
R
O

I
Lipid triglycerides fats
FATY ACIDS
def long
: chain
C- C
carboxylic
-
C - C -
C
acid
-

COOH

1
classification of F. A

1. Based on no.

of double bonds


Saturated FA > no double bonds
• Unsaturated FA
z
MUFA : 1 double bonds
PUFA : 1 double bonds .

2. Based on nutritional requirement -


-
-
-

c-)
r

* Essential F. A. :
:
I most essential
#
no .

of double bonds :D
-omega
1. LINOLEIC ACID 1.8 : I 4,12¥ : no
no .
ofcarbons positionofdb prega
LINOLENIC ACID 18 :3 (9112,1-5) :
-oregant
.

2. wz

3. ARACHIDONIC ACID 20 : 4 ( 5,8 , 11,14 ) : we


Classification of LIPIDS :

LIPIDS

simple complex
1
volgate
I
( FAT Abe ) (FAtAkt PCH
)
Protein

Fpd
z
i.
Phospholipid
Glycolipids
waxes
Oils
fats a.

3 3.
Lipoprotein
(FAT Glycerol) ( FAT Alcohol Except
Glycerol)
PHOPHOLIPID
FAT Alcohol +
Phosphate
< >
FA 1- Glyurdtp FAT
sphingosine + P

glyuropholipid sphingo phospholipid


.

F-
spingomydin
Neiman Pick

dlsbpingomydinaseYVGLYWLIPIDFATA-lc.tc.t
-

L
s
FAtAkot monosaccharide FAtAk 1-.

Ol&igosaccharides
3
Cerebroside Gangliosides .

TAY SACH 's d- hexosaminidase


< S
FAT Atetbiu FATA let Galactose SAND Hoff 's B- hexosaminidase
.

I &

Gluwurebroside Galactoarebroside
GAUCHER
's FABRY dls Galaetocaebsosidase
's d-

dls ghewaeebsosidase KRABBE dts B- 's "


✗ ,
,
LIPOPROTEINS •

Types of LP 's :

Chyhomiown

" >

VLDL

lipid t
Apoproteins IDL

LDL
ApoA Apoc

TG
cholesterol HDL
ApoBqgApoE

Metabolism
of Chylomioons :

dietary fat exogenous


{ fun yqÑRBa Peripheral tissue Adipocytes


-

g. g.
.

"tApoÉ
, A- pot 1
And .

d-
e + receptor
a
¥i¥÷÷
Chylomiowns
Bloodhound
}

①-n_ Lipoprotei
Es ↳PL -

TG ApoB.az
and Lipase
Aport
.

Chylomieron Remnant
Metabolism
of VLDL : -

fat
Endogenous

!mxqq¥poB
>

synthesize
Kei *
Chol .

-
Apoc "
^

Blood[) }
e " Ph

VLDL

•\TqtApoBi00 And
Apo E
.
-

L VLDL remnant IDW

TG -

Apo Bioo
Chol .

LDL

HDL
AMA
Tg
-
:
Apo F-
Chol
-

Apoc
.
-
Composition of LIPOPROTEINS
cholesterol ? LDL
Name Apoproteins Lipids { c-

c-
has highest
has highest TG ? VLDL
culgamicrons

e)
Chybmioons Apo Base Esc,

VLDL Apo Bioo ,


C- ,C

In Apopaoo ,

LDL Apo Bioo

HDL Apo A ,
E ,C

Functions
of Apo proteins

}
ApoBA8
Apo Bioo interaction
of lipoprotein c-
Receptor
Apo E

Apoc > Activation


of LPL (lipoprotein lipase
Apo A > Activation of LCAT ( lecithin chd .

Acyltransferase)
LIPID METABOLISM

Synthesis of cholesterol

AÑgAf+ Acetyl GA

Auto
acetyl CoA

acetyl bA→ v
* *
1 RLE
HMGGA
HMGCoAReductase_mm)
&
inhibits @ 0A)
Statin
i
Y
Mevalonate

↳ renoid Units

Squalene

Chibstrd
cholesterol
degradation of :

CHOLESTEROL

Vit?D
' >

Steroid hormones Bile Acids


->
Mineralocorticoid
Glucocorticoid
, ,

Testosterone , Estrogen Progestione


,
Synthesis of F. A :
cytosol

duty ↳ A ¥ÑmwÑ¥ÑÑonyl
CO2
↳A
tatty acid synthase
complex
>
fatty Acids

Degradation of F. A : -

LIPIDS

lipolysis
f- A GLYCEROL

B- oxidatn
of
Fatty Acids

Acetyl Co -

ketogenesis

ketone Bodies
B- oxidation of F. A .

I
3
stages
Activation
g. -

Cytosol
2.
Transport
[carnitine Shuttle] *

B- oxidation Mitochondrial Matrix


proper
a. -
e

I. ACTIVATION :
cytosol
FACPALMATICAUD : 164¥
-
c -
c -
c - c -
c -
COOH

LATP
>
Acyl CoA Synthase
v

-
Acyllott
C- C- C- C- c- CoA

II. TRANSPORT : Carnitine Shuttle

"%m.{[
matrix
cytosol
"& " " Gomi" carnitine
^ '
Acyllott
CAT -1 CAT -2 (carnitine acyl
transferase)
CoA
' s Acyl → Acyl CoA
carnitine carnitine
II. B- oxidation Proper : Mitochondrial Matrix

>
FAD
Ayllo A
Oxidation Y
FADH2 <

Delta 2 trans CoA


enoyl
Hao >
Hydration ↓
V

B- Hydroxy Acyl CoA


NADT
Oxidation X
NADH + Htc
v

B- keto
Acyl CoA

*
Cleavage
' >

Acyl CoA
Acetyl(CoA
2C)

ENERGETICS 8

Dalmatic Acid @ c) B-oxidation Propes= 7 times

gain of
Net ATP : 106 TFADH x 1 5.
=
10 5
.

5 17 5
NADU2 X 2

-

- =
.

- Acetyl (Krebcycle)
8 CA x10 =
80
1
- -
-

108
< Activat 2
-
Note
End Product
Fatty Acid

Even Chain
fatty acid Acetyl CoA

Odd "

(OMIT)
" •
Propionyl GA

KETONE BODIES
1. Acetoacetate

2. Acetone

3.
B-
Hydroxy Butyrate
KETOGENESIS
z Retone Body Acetyl CoA +
Acetyl CoA

i. VK.rs : acetoacetate
D. location : liver Mitochondria
D. K B. cannot be utilized

Acetoacetyllott
.

LIVER & RBC


by .

Acetyl CoA →

AMIGA
Acetyl bA←✓
Acetoacetate
>
<

Acetone B-
hydroxy
butyrate
21st '
21
July

NUCLEIC ACIDS

DNA RNA

C- rely
DNA
charged)

§{
-

double stranded
nucleotide
-
helical
strand :
-

polymer of
complementary pairing
-

-
anti
parallel
-

IN
Nucleotide in
i.
Sugar
P : poi
'
2.
gp
-
.

3.
Nitrogenous Base
5

Sugars 3 CH o


q p

deoxyribose sugar ¥4 3 I
Base
Nitrogenous :

major
Purines
Pyrimidines
adenine
cytosine
- -

guanine thymine
- -

minor

hypoxanthine
-

Xanthine
-
Uric Acid

Nucleotide STPTNB Nucleoside STNB

O O

NB
P 4

I NB
K g s •

2
3
NB nucleoside nucleotide
NBTS NBTSTP Adenosine

adenine Adenosine AMRADP, ATP Monophosph ats

pay
-

guanine Guanosine

GMP.CMP.
GDP.GCTPpyycytosi
DP.
n el
CTPthymine.MU
Thymidine
y ti d . m e TMP.TDP.TW
1
Uracil Uridine UMPGUDPGUTP
@N*)

Structure of DNA :

-
Watson Crick DNA
B- DNA µ bond

zgnpzi.ms
-
: weak

P ↳
<
-
s -

A
Phosphodiester o

MtMP'(break)
bond P -
s + A Mph .

I
-

S
-

-
bond
p
strong P -
s
q
-

C S
Hemp
-

-
p
.

p.ph P -
S A T
-

S
1
-

-
p
-

does not P -
S c
G
-

-
S p
break 3
. -

51
Chargeftp.s Rule
No

of purines of pyrimidine

.
=
no .

At G = C + T

2.
Complementary Pairing
A

:)
pie

÷
Mr T

A- In . a DNA
, if G. = 15% ,
then 1- =
?
A = -1

G=C

Of .
In a DNA ,
if A =
75%
z
,
then 1- =
?

not
possible
1
does not exist
for could be

ss -
DNA
Types of DNA

A B C D E 2
& I
0
B- DNA
Watson-Crick DNA
I

As • ds

Righthanded helix left handed helix



Denaturation / Renaturation
Melting Annealing
vice versa
Atemp9pH
ht bonds Break
-

Organization of DNA
Mmmmm

⑦hgT
chromatin
to
mmr

ou v
nucleosome
3
Einar v


histone protein Linker DNA

DNA
+
bats
H i
,
Histone Protein

core : Octamer

of histone
protein
Itza Hz
2
copies
.

HZB Hq

'

+ I + !! +

DNA : -

rely charged : dlt Poof gp .

Histone : +
rely charged : A
Arginine Lysine
&
NUCLEOTIDE metabolism
double
ring

Purine
Ring -

Heterocyclic
~

CO2

i
> Glycine
6 Mt ?
Aspartate µ ? '

is THF
-

, ,
gas
① I
1
Trip ,
Yes
,
,
am
NÑ a

Glutamine

synthesis of Purine

Synthesis Salvage Pathway


< •
Denaro

H&¥Ribose 5- P

IÑP
AMÑ
>

Gmp
IMP ? Inosine Monophosphate
P
th

hypoxanthine

De Novo Ribose

synthesis .
5- P

ATP
PRPP
synthase
AMP <

N
**
Phosphorihosyl
Pysophoshateprpp)
Amidotransfoau
Glutamine
N

Phosphoribosyl
Amine -> NHc

v
Ni Aspartate
f Caco -
THF

I NzNs
I
-

Glutamine
y Calvin -

Glycine
G- Coz
IMP
"
"

AMP GMP
Salvage Pathway
Adenine -

Phosphosikosyl
PTPÉTRP + Adenine transferase
sppi
> ⑨YpÉf④
PRPP
AMP

P-p¥Éµ× HGPRT
'
>
"
p-
"*

Papp PPI
Imp

'p①
P P

PipG
- -

HGPRT
+ Guanine →
,

ppi
GAP

*A
4C Lesch
Nyhan Synd .

HGPRT [Hypoxanthine Guanine


eny
.

def : Phosphoribosyl Transferase)


elf :
Neurological
GOUT
deficit
Mutilation Charm]
· self
Joint Pain
Degradation of Purines :

AMP GMP Guanosine IMP Inosine

Monophosphate Monophosphate

:
Adenosine
Monophosphate

HYPOXANTHINE
xanthine oxide <
-0
Allopurinol
Xainthine
Xanthine Oxide

*
End
product : ÑRK ACID

Pseudo Gout
CK GOUT 3
??
crystals
3
Rosphlgestals
c-
Calcium

hypomania diaydsate

MSU
crystals
°

Monosodium
}
Urate
Cystals needle shape Gouty
Arteritis

Rx : Acute GOUT : NSAIDs


Chronic Gout : Allopurinol
MOLECULAR BIOLOGY
Central Dogma of Molecular
DNA Replication DNA
biology DNA
polymerase
Transcript
"

RNA polymerase

m -
ÑNA
MIRNA
Translation}z
Proteins

Replication
.

: -

DNA DNA
def : DNA
dependent
>

DNA
polymerase
location : Nucleus
?
when : S -

phase of cell
Cycle }

r ye DNA-A ssotein
I

} separates
Hemi Conservative
-

Process
1
half of parent DNA will be conserved .
the strands
* DNA A Protein :
separate *
-

Protein
*
Single Strand Binding :
stabilized the
single strand [5SB'S]
* Helicases : uncoil helix
~ Topoisomerase
-
I
Nick 's
*
Topoisomerase : relax
supercool by Seating
XII/DNA gyrase
- -

I
DNA A
-

T I -


T -

I DNA
gyrase SSB 's

UNM Helicases > ÑÑ

Bag
<

- /
Replication e

I
-
DNA-A Protein 3
Topoisomerase
'

Reading
'

3 → 5

DNA
polymerase III.
-

New Strand 5
'
> 3
'

RNA Primer

LEADING : towards replied" fork


%
-

'M
strand continuous
Replicating Bubble -
.

Primrose
>

h.GG C
TA
I'D
-
T C C
G AT
'
5 > 3
'

Replicating fork
imam'

J
'
3

5
'
D
?
z

'T
5 31

LAGGING strand :

from optical fork


-
"

away

•discontinuous

1
OKAZAKI
fragments

RNA Prima :

formation
removal
:
:
Primrose
RNase H
I
Gap filling : DNA
polymerase -

ligation : DNA
ligase .
DNA Polymerase
Prokaryotes Eukaryotes
DNAP ✗ -
: Primrose
DNA I : gap
filling
-

DNAP B : DNA repair


-

DNA -

I: DNA
repair

&
DNAP-8 :
Replication of Mito DNA
DNA -

III :
leading
lagging strand
}
DNAPS
leading &
lagging strand

DNAP E -
PROTEIN SYNTHESIS
DNA

transcription
hn? RNA Chetwonuclear RNAS

nucleus *A
post-transcriptional modification
cytosol v

m -
RNA

I
m -
RNA

Translation

ein

}
Post-translational modification
TRANSCRIPTION

RNA

def : DNA
DNA -

dependent
>

RNA polymerase

location : nucleus .

Elongation Termination
zi Initiation 5
,

"
if ¥
"
TATAAT - - - - -
- -

go

2¥&dequen% C G G CAU
p >
51 3'

Region 31
'
5 Promotion

PROKARYOTES
losequnce TATA Boxthibnow
'
f-
Reading
'

Box 5
-
:3 >

(Rho)
dependent
TATAAT ' termination
RNA ; 51 > 3 z .

-35
sequence g- protein stops
RNA
formation
TTGACA remove RNA
A- = i
f- independent
EUKARYOTES ✓
termination
Box U
-25
sequence Hogness 2g '

forms hairpin /bend


TATA RNA

TO to -80
-

sequence
CAAT Box
CAAT
RNA POLYMERASE

XX
:

PORKARYOTES EUKARYOTES
}
RNAP I RNA
RNAP -
=
8-
except 5s subunit

1
subunits RNAP I hn RNA, RNA
:zgµµ
-
= - m -

RNAP -

II = t -
RNA 5s RNA

factor : •
factor
1
It PROMOTAR
recognise region

Post-transcriptional Modification of
his - RNA
I " "

{☒ l 1
' ' ' '

g. q e
z@a-a-A-a.a.a
ñcapping-hmu Poly-A?ailmwM
7-
1 Introns :
non-functional
methyl Exons :
Guanosine functional
ÉPLICING : removal of Intron &
ligation of Exon
I
done
by : spliceosome snRNP 's
Snurps .
GENETIC CODE CODON
which

codon combination 3 nucleotides


def : is a
Acid
of e
8 codes

for an Amino

AUG Methionine
egg
: codes
for

No 64 Codons
of CODONS =
.

A "
0
pm
G
(4)
'

(g) =
4×4×4=64
'

Py u

mRNA UAAIUAG/UGA
AY ,
G
, " t
' 31
5
f
1
Initiation Codon Terminal Codon
"

1
Stop Codon non sense
-

codon
z
.

i. e start codon C
i.
estop .

I
Characteristic

feature of CODON

1. Universal same codon codes


for same Amino asids all
species
in

2. Commaless and
non-overlapping AUG )
Specificity Unambigous ( Met
'

3. > .

a.
Degenerate codon { than 1 codon }
AA can be coded
by more

3 I

RIBOSOMES :

Eu
Promos 80s

50s 30s 60s 40s

RNA :

stranded
single


linear N -
N -
N -

N -

strand :
polymer of nucleotide

i.
Sugar : Ribose

gp Poa
2. P -
: -3
Adenine
3. NB Pu :X Guanine

Cytosine
Py :-, Uracil

Types of RNA : .

RNA -
Funchon
Transfer of
-

RNA ( abundant )¥ of
1. r -
most
Amino feid
2. m -
RNA

3. f- RNA (smallest)
q
tiniest

f- RNA :

structure :
cloverleaf shaped
Amino Acid

DHU arm

qE±mam
Anti Codon
Arm
-
-
-
TYC
variable Arm
Arm
Post-translational Modification of Collagen
DNA
Collagen : Hydroxylation
I

RNA
at
lysine &
proline
m -

OH odt
AA AA AA AA AA
lysine AA AA Proline AA
- -
-
- -
- - -

v lysyl Prolyl
Proteins hydroxylase Vitt hydroxylase viii. c.
.

Co-enzyme
->
collagen OH OH

OH
wring
on

Triple helix -

tysyl
Oxidase cu

Note : is .
DNA

MYRNA

Hotin
no defect defective
%fei.nu i Ubiquitin
"

Protein
folding -

¥ "ÑHAPEROpvEs
"
^^ ^
Proteasome
Structure Proteins
2.
of
40 linear

✗ Helix &
¥20
-

/
B- pleated sheets -==
3° 3. dimensional

40

a.
Blotting techniques
Northern blotting RNA
Southern " DNA
Wiston "
Proteins
VITAMINS -

(3-59)

Lipid Soluble Water sdube


ADEK Vit B complex-

vt c -

Vit A
-

Retinol Gttcohd)
chemistry ii. OH
-

2. Retinal -01+0
Aldehyde)
3.
Patino :c acid coat
-

( carboxylic Acid)

Good source : Beaudine digestion > 2. Retinal


vita Vita
pro
-

Best source : Hdlibut Fish liver oil .

Funtime : i. Vision Rhodopsin ( opsin +11 Cis Retinal)


-

Epithelium Gandhi & differentiate


"
a.

3.
Reproduction
Oxidant
Germ Cells >
Sperm
a. Anti -
deficiency diseases
1.
Nyctalopia -

night Blindess

2.
Xeropthahnia -

Complete Blindness
3. Keratomalacia corneal -

ulceration
a.
dry rough & scaly skin
,

5
Infertility
Vit - D

Vit Di Calciferol
2 Ergocalciferol
3 Cholecalciferol .

Synthesis & Activation


liver of vittles =
Akin
kidney
- -

= i
hunkering
7-
dehydsodobsterd
v.v
rays
cholecalciferol

•÷m①
CC

-
OH →
25 hydroxylase
25
hydroxy
-

UCC
cholecalciferol
picot 25 Hit
-

actircatn
of Vitas >
1- ✗
hydroxylase
-

OH >

1,25 dittcc Vit Dos Cakitriol (active form)

Functions
the blood calcium level
a.
Regulate " ^

ÑW
dl
r n 9-
11mg .

§
"
" "
it " ⇐
<

D
Ppabsospt Kx
,
Vit -

Cata

a

Difeciency disease :

>
Children :
Rickets
Vil:D
>
Adults :
Osteomalacia
Vitter
Vit ks
Phylloquinone} natural
°
.

kz : Mena
quinone
Ks : Menadione synthetic
&
water soluble

Functions :
Lut
8- Carboxylate


at

vikk
Glutamate
required for
activator

dfd :
BlP.T.lt
eeding disorder

Vit f-
-

active
form : d- tocopherol
Anti oxidant
n

fund :
-

dld :
neurological disorders
Vit B Complex
Name
Coenzyme did
Enzyme
VI. B. Thiamine TPP tipoioacid Beri Beri

}
1. -
PDH - -

-
✗ KGDIH TPP
CoA
-

BCRDH
NAD

☒Cw3 FAD

Transkilobase TPP ,
> Wernicke -

KorsakoffSym
G : Global confusion
0:
opthalmopkgia
A : Ataxia

2. Vit Bz Riboflavin
-

FAD -

dehydrogenase Chidosis
.pµµ AngularStomatitis
Glossitis
3. VitBz Niacin
Nicotinic Acid NADT - "
Pellagra
Tryptophan Nitz
-

Dementia
60mg
1m Cphohtsensitive) -
Dermatitis
-
Diarrhoea
3
Death .
a. V-d-Bspanb-thenic.CA Burning foot
-

synd .

Vit Bg ALA
5.
Pyridoxine PLP 1.
synthase -
Sideroblastic
(Pyridoxal Chemosynthesis) Anemia
phosphate)
2. AST ALT
Aspartate (transaminases) Alanine

i. VitB , Biotin i.
Pyruvate carboxylase Allopecia
Biotin 2 .

PropionylCoA " Chan loss)


Vit H
Acxtyl CoA
"
-

3.

mcc >
Bqv Consumption of
Raw Eggs

Egg While
}

Bio-%{hnw)
" "

AVIDIN
+

3
Excreted
Urmocysteine Methyltransferase
Vit Bis
8.

Cobalamin
Vit Biz
"
i. MMT
MMC mutase

Mlgaloblastio
Anemia

Methy/Malony/COA •
Peripheral neuropathy
9. Vit Bq
Fdicidcid
food
DHF

1. } folate Titan
'

folic acid > > Diff > THF DNA and RNA
,
111-2 H2

{→ in

Absorption
Jejunum
did :
Megaloblastic
NTD
Anemia Y

's
Vit .
C Ascorbic Acid
functions : i. Posttranslational modification of collagen
a.
Absorption of Irun
3. Anti oxidant
-
.

dd :
scurvy
Note : Antioxidant
{
A C C-
I
most
potent
ENZYMES
A
def : velocity of Reaction
nature ? Protein t Won Protein
3 3
Apoenzyme co -

/
enzyme
Co
factor/
-

Prosthetic
group
.

Factors ?

Enzyme
EA
p
i. Conan
of E ✗ V s >

substratt Product
in .
Conan of s ✗ v

* Conon of Port Bell


shape graph
4. Temp > mas .

5. PH

of "
React I

d) I
,

t
optimum temp
temp @ 0-450)

of "
React I

d) I
I.

)
p 7 19
pm
PM
optimum
Michaelis Menten Graph .
: Ym3 )
constant
(8) V m.mg
.

or

concentrate
man Vel
of substrate at Half
.

Imax -
-
-
- -
-
- - -

vet of
React
" parabolic
Vms
-

(4
-
- -

,
-

i pin

i. A

Is]
-
Enzyme inhibition
Competitive : Vmax same Kmt
g. Reversible Non-competitive Vmaxlv
-

knnsame
2. Irreversible

Isoenzymes ok -3 Creatine
Kinase
: LDH -5 Ck mm : skomsl
eg
-
.

Lactrite
Dehydrogenase
}
↳ Hi OR BB
Heart
-
:
Brain
LDHZ
* MB Heart
CK
Brain RBC
spleen
:
LDH }
.
-

LDH
LDHstgsk.ms
a
.

LDNz
Normally
LDD, >

-DA , <LD42
MI
Haemoglobin :

Non protein protein

structure of theme

4
Pyrrole ring M

• I :[ :-#I
r

Piolo
porphyrin LET
home
?
feet >

Home
home
¥¥É¥¥jÉÉɱÉÉÉt
Gabin
{{ "
chain

B X or B
HEME
Synthesis of :

Succinyl GA t
Glycine
Ala synthase
pLpRhH
Vv

8- Aminolevukeniic acid
G- ALA)
lead/Pb H2O

e
'ALA -

dehydratase

PooÑobilinogen(PB4)
V

Nnoposphyrinoqen
I

Copwphyrinogen
I
Piutophyninogen
Proto Gysin
/ Foowchelatase
-

head Pb Fia _ j
>

HEME
DEGRADATION of HAEMOGLOBIN :

Hemolysis

.!
① >
rise
420days) hemoglobin
(will
release out)
EDIE &Ñ
spleen
i. :
Bilirubin A. A

conjugation MATEY Ctfeutiliged)

°hddd
UDP
-

Glucosuria>
Arid v
°

Bilirubin
diqluooyonide > .

Soluble
Coyiucalid
-
.

won :-|
.

i. F-
.

I 1
I ✓
I 1
1 I
'

'
L -
- - - - - - -
d l

p-z.eir-w.mn#-.ui -isiein
'

Urine
- - - - -

>

Sterabilin → Stool .

v
Biochemistry LMR - Dr. Mohammed Azam
Pathway Site Rate limiting enzyme
CARBOHYDRATE METABOLISM
Glycolysis/Embden Mayerhoff Cytosol Phosphofructokinase-I (PFK-I)
Pathway (EMP)

Krebs Cycle /TCA cycle /Citric Acid Mitochondrial Matrix Isocitrate Dehydrogenase
Cycle

Gluconeogenesis Liver & Kidney ( Cytosol & Fructose 1,6-bisphosphatase


Mitochondria)
Glycogenesis Liver & Skeletal muscle (Cytosol) Glycogen Synthase
Glycogenolysis Liver & Skeletal muscle (Cytosol) Glycogen Phosphorylase
HMP Shunt/ Pentose Phosphate Cytosol Glucose-6-Phosphate dehydrogenase
pathway (G6PD)
PROTEIN METABOLISM
Urea cycle/Ornithine Cycle Liver (Cytosol & Mitochondria) Carbamoyl Phosphate Synthase-I
(CPS-I)
LIPID METABOLISM
Beta oxidation of fatty acid Mitochondria Carnitine Acyl Transferase-I (CAT-I)
Ketogenesis Mitochondria HMG CoA Synthase
Fatty acid Synthesis Cytosol Acetyl CoA Carboxylase
Cholesterol Synthesis Cytosol HMG CoA Reductase
NUCLEOTIDE METABOLISM
Purine Synthesis PRPP glutamyl Amidotransferase
CONSISTENCY is mantra of SUCCESS - Dr Mohammed Azam

Disease Enzyme Deficient

Carbohydrates

Type I/ Von Gierke's Disease Glucose-6-phosphatase SEVERE Hypoglycemia, Hepatomegaly &


Renomegaly, Lactic acidosis,
Hyperlipidemia, Ketosis and Hyperuricemia
(Gout).
Type II/Pompe's Disease Acid Maltase/ Lysosomal glucosidase Cardiomyopathy, hypotonia & systemic
findings lead to early death by 2 year.
Type III/Cori's Disease/Limit Debranching Enzyme Similar to TYPE-I but milder symptoms &
Dextrinosis normal blood lactate levels.
Type IV/ Anderson's Disease Branching Enzyme Hepatosplenomegaly, failure to thrive in
early infancy & progressive cirrhosis (death
usually before 5th year).
Type V/ Mc Arlde's Disease Muscle Glycogen Phosphorylase Painful muscle cramps with strenuous
exercise & muscle glycogen abnormally
high.
Type VI/ Her's Disease Hepatic Glycogen Phosphorylase Hepatomegaly.
Essential Fructosuria Fructokinase Fructose appears in blood & urine.
Fructose Intolerance Aldolase B Jaundice, vomiting, seizures, lethargy.
Hepatomegaly.
Galactossemia Galactose-1-phosphatase uridyl Jaundice, vomiting, seizures, lethargy.
transferase (GALPUT) Hepatomegaly & Oil drop cataract.
Mental retardation.
Protein's

Phenylketonuria (Mousy/Musty Phenylalanine Hydroxylase Hypopigmented skin, Blonde hairs,


odour) Intellectual disability, microcephaly, seizures
& mousey or musty odour.
Alkaptonuria /Black Urine Disease Homogentisate Oxidase Urine turns black on prolonged exposure to
air; Ochronosis (bluish-black connective
tissue, ear cartilage & sclerae.
Maple Syrup Urine Disease (Burnt Branched Chain Keta Acid Vomiting, poor feeding, urine smells like
Sugar Odour) Dehydrogenase/Decarboxylase maple syrup/burnt sugar.
Lipid Storage Diseases
Niemann Pick Disease Sphingomyelinase
Gaucher's Disease Beta-Glucocerebrosidase
Krabbe's Disease Beta- Galactocerebrosidase
Fabry's Disease Alpha- Galactocerebrosidase
Tay Sach's Disease Hexosaminidase A
Sand Hoff's Disease Hexosaminidase A & B
Nucleotide's
Lesch Nyhan Syndrome HGPRT Hyperuricemia & Gout, Intellectual
disability, Self mutilation and red/orange
crystals in urine.
Gout Hyperuricemia Needle shaped MonoSodium Urate (MSU)
Crystals and Gouty arthritis.
CONSISTENCY is mantra of SUCCESS - Dr Mohammed Azam

Amino Acid Special Product


Tyrosine Melanin, T3, T4, Catecholamines
Tryptophan Melatonin, Serotonin, Vit B3
Histidine Histamine
Arginine Nitric Oxide (NO), Creatine
Glycine Heme, Purine ring, Glutathione, Creatine

Inhibitor
Flouride (NaF) Enolase ( Glycolysis)
Barbiturates(PhenobarbitONE) and RotenONE Complex-I (ETC)
Malonate Complex-II (ETC)
Antimycin, British Anti Lewisite (BAL) Complex-III (ETC)
CO, CN, H2S Complex-IV (ETC)
Oligomycin Complex-V (ETC) - ADP to ATP Conversion

Classification of Amino Acids


Aliphatic Amino Acid Glycine, Alanine, Valine, Leucine, Isoleucine
Hydroxyl group containing Amino Acid Serine, Threonine
Sulphur containing Amino Acid Cysteine, Methionine
Acidic Amino Acid Aspartic Acid, Glutamic Acid, Asparagine, Glutamine
Basic Amino Acid Lysine, Arginine, Histidine
Aromatic Amino Acid Tyrosine, Tryptophan, Phenylalanine
Imino Acid Proline
Essential Amino Acid Tryptophan, Valine, Threonine, Isoleucine,Leucine, Lysine,
Phenylalanine, Methionine (TV TILL 8 PM)
Semi Essential Amino Acid Arginine, Histidine
Ketogenic Amino Acid Leucine, Lysine
Glucogenic and Ketogenic Amino Acid Tyrosine, Tryptophan, Phenylalanine, Isoleucine
Glucogenic Amino Acid Rest All
21st Amino Acid Selenocysteine (UGA)
22nd Amino Acid Pyrrolysine (UAG)
Fatty Acids
Essential Fatty Acids Linoleic Acid, Linolenic Acid, Arachidonic Acid

Fat Soluble Vitamins


Vitamin Chemistry Function Deficiency
Vit-A Retinol Vision ( Rhodopsin : Opsin Nyctalopia
Retinal + 11Cis Retinal) Xerophthalmia
Retinoic Acid Normal Reproduction Keratomalacia
Normal differentiation of Dry & scaly skin
Epithelium Bitot spots (Keratin debris;
Antioxidant foamy appearance on
conjunctiva)
Vit- D Vit-D1(Calciferol) Intestinal absorption of Rickets in children
Vit-D2 (Ergocalciferol) calcium and phosphate. Osteomalacia in adults
Vit-D3 (Cholecalciferol) Bone mineralization.
Reabsorption of calcium&
phosphate from kidney.
Vit-E Tocopherol Antioxidant Neurological disorders
Hemolytic anemia
Vit-K Vit-K1 (Phylloquinone) Activated form acts as Neonatal hemorrhage with
Vit-K2 (Menaquinone) cofactor for the increased PT & aPTT but
Vit-K3 (Menadione) γ-carboxylation of normal bleeding time.
Glutamate for clotting
factors II,VII,IX, X and
Protein C&S.

Water Soluble Vitamins


Vitamin Cofactor Enzyme Deficiency
Vit-B1 ( Thiamine ) Thiamine Pyrophosphate Pyruvate Beri-Beri
(TPP) Dehydrogenase(PDH) Wernicke-Korsakoff
α-Ketoglutarate Syndrome
dehydrogenase
Branched chain ketoacid
dehydrogenase (BCKD)
Transketolase
Vit-B2 (Riboflavin) FAD & FMN Dehydrogenases Chielosis (inflammation of
lips, scaling and fissures at
the corners of the mouth)
Magenta tongue
Seborrheic dermatitis
Vit-B3 ( Niacin/Nicotinic NAD & NADP Dehydrogenases Pellagra
acid) - Dementia
- Diarrhea
- Dermatitis (Casal’s
Necklace)

Vit-B5 ( Pantothenic Acid ) CoA ( Coenzyme A ) - Burning feet Syndrome,


dermatitis, alopecia
Vit-B6 ( Pyridoxine ) Pyridoxal Phosphate (PLP) Transamination (ALT&AST) Sideroblastic Anemia
Heme synthesis ( ALA Conculsions
Synthase) Peripheral Neuropathy
Decarboxylation reactions.
Glycogen Phosphorylase.
Vit- B7 ( Biotin/Vit-H) Biotin Pyruvate carboxylase Alopecia
Propionyl CoA carboxylase Caused by long term
Acetyl Carboxylase antibiotic use or excessive
ingestion of raw egg
whites(AVIDIN in egg
whites avidly binds biotin)
Vit- B12 ( Cobalamin) Vit-B12 Homocysteine methyl Megaloblastic Anemia
transferase (HMT) Peripheral Neuropathy
Methyl malonyl CoA Inc. serum Homocysteine
Mutase (MMC Mutase) and Methylmalonic acid.
Vit – B9 ( Folic Acid ) Converted to tetrahydrofolate (THF), which is important for Megaloblastic Anemia
synthesis of nitrogenous bases in DNA & RNA Neural Tube Defects
Inc. serum Homocysteine.
Vit – C (Ascorbic Acid) Antioxidant SCURVY (swollen gums,
Iron absorption poor wound healing, easy
Post translational modification of collagen bruising, petechiae,
(Hydroxylation of Proline and Lysine ) hemarthrosis, corkscrew
hair)

-Nucleosome is formed by DNA looped around histone


octamer(H2A, H2B, H3 & H4).
- H1 binds to linker DNA.
-DNA has negative charge from phosphate group.
-Histones are positive charge from lysine and arginine.

Nucleosomiz

ET

Pathological 2,4DNP Aspirin


TUncouplett
Physiological Thermogybownfa
Thyroxine
FreeFattyAcid
Bilirubin
EnzymeInhibition

Boat BATE

Isoenzymes

tRNAs

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