BIOCHEMISTRY

BONUS-BTR
ENZYMES U
of gentamicacid.A
carbonylation

Enzyme classification
sensperbola)
EC -

③
Oxidative E8-I
~V

(Be APT
decarboxylase? B1
in
Menter.
&
-
O, dissociation
All kinase/ phosphorylase/
EC 1 ocidation/reduction carboxylase use Mg except Signateit t

(phosphate mostly) (from Pil pyruvate kinase-K

A cater edge is
(understandcalmesw-come,
ofKen
ati t

50%ver
is
salviation
in

(from ATP/GTP)
c ome at
substiate
EC 2
Synthase except: 50%
Umax

(10.5/p50)

NO synthase 1 Left strift-Iaffinity

(0, release (d)
EC3 Glycogen synthase y
Left Loves i

Citrate synthase I
(O release 44)
ATP synthase 3 Rightshift-I affinity
EC4 B Love less i
Hydratase, Aldolase, Synthase B
B
Lyase Carboxylase: Mg
EC5
A ATP
B Bioe
-

E26 9
Synthetase, GT
C We
Carboxylase Decarboxylase: Bo

7th class: Translocase

Kcat/Km: Enzyme efficiency

MC5232
Glycosaminoglycans
Acidic sugar: Glucuronic acid, Iduronic acid
Aminosugar: Glucosamine, Galactosamine
Negatively charged
③
Absorbs water

Most abundant: Chondroitin

sulfate
GAG with no protein linkage:
-

GAG with no sulphate: My

acid
almonic

Cell migration during morphogenesis, wound repair:

Cartilage: Chondroitin acid
B sulfate & Thyalmonic
GAG with no uronic acid:
B
Revatan
sulfate
Corneal transparency:
Sclera:
Atherogenic (LDL binding):
Dermatan
sulfate
LPL on endothelial surface:
Plasma membrane receptor: Hepasan sulfate
GBM charge selectiveness:
 Types of lipids and lysosomal diseases
PHOSPHO-LIPIDS GLYCOSPHINGOLIPIDS: No Po4

Glycero-phospholipid: Sphingo-phospholipid Cerebroside : Ceramide + Monosaccaride

1. Cell membrane: Globoside : Ceramide + Oligosaccharide
Phosphaditic acid Glycol FA+ POG +
Sphingomyelin Ganglioside (GM1, GM2, GM3): Globoside + NANA
Phosphatidyl choline Lecithin
(Most abundantin (Ceramide + choline + Po4 ) B
GM1- CT receptor
surfactant)
P. Ethanolamine Cholera

Cephalin Jonin R
APLA ⑨
2. Inner mitoch membrane, antigenic acid lipase cat

DiP glycerol cardiolip 1 Ab+ ⑨ syndrome)

in Barli
~Bladrenal diarrhea (vid 23, Ed6)
Wolman's disease:watery
green
Diphosphatidy
3. Apoptosis Ceramidase defective:
[Link] Farber's disease RA
mimics
Hexosaminidase A Tay Sach
4. Secondary messenger Hexosaminidase A, B sandhoff

[Link] IP3DAG pathway (and messenger)

atass
says self Fabry's
XLR
a
galactosidase
No CRS, No MR: Fabry's MLD

Gauchers
Icaception:Type II Gaucher's
-

psendo chemyred spot) Gauchers B glucosidase

B galactosidase
Krabbe's

NPS
sphingomyelinase
 4
S M
 pathway
catabolic (but I insulin-in
well
fed state) shongestactivatis butnotdirectsubsticti
(Acchys (A)

ENERGETICS: ATP
used
Fasting (16-48hrs)
Aerobic glycolysis- 9 -
27=

Liver-kidney
Anaerobic glycolysis- -2 2=

Pinsulin regulatesGlucokinase Substrates:

I NADH X)
1) Alanine (Cahill)
2) Lactate (Cori)
bresedoneanaerobiat
itGlygen c the g
3) Gycerol (TG)
4 1: 3 ATP
4) Propionyl CoA (Odd chain
... -

7 RE
t
ATP
need
FA) PMS

DFK-biOn NOT ACETYL CoA

Fanc 8-16 his:
Glycogen
16-48 his:
Gluconeogenesis

carbohydeses ->

fat
becomes
generates
NADH
↓
(2) Scetys CoA-encess
cholestial
& ATP lost 2.5 5
2x
ATP
=

9ed iscarbohydrate
fat
ATP StP
5- 2 2
+ = recover

needs GTP
LATP, SLP

Entire
pathway
reversible
except & Malate-asparate shuttle
3 inversible
'MAO

mitochondria
in
needs ATP, Biotin, CO2
Krebs cycle
Aerobic total- 7 5 +

20
+
32
=

(Glycolysis + PDH + TCA)

&NADH
2.5x3

Amphibolic pathway: catabolic + anabolic

Anaplerotic reaction:

Succinyl coA-Heme -decarbonylation. Be

Alpha keto glutarate-Glutamate, GABA

Citrate-Fatty acid synthesis
RLE

BI

Fe
7.5
x 2.5:
3 NADH

1 FADH x 1.5 1.5

=

1
=

1 GTP
SLP
Complex 2 10 x 2

Inhibitor: Malonate 38
=

MSD a
is FelIron) Man
 gluonesgenesis
Glycogen metabolism
X
liner, kidney
fally
glycogenolysis
hypoglycemia Lactic acidosis, ↑ Retogenesis,
4 micemia
i cholesterof, HMp
palhinay
-> ↑ perloses -

~for over 1 exercise)

-glose
in

fights flight
glycogenolysis himer, Mustaphataxi
-

for body
e
~ ist
&D
Gas
AB
amylopectin

-
26 gesidue
dephosphorylated
(RE)
phosphorylated

1,6 ③
2-
.....
B

2-1,4
↓ Glycogen
primes)
Glycogenin (polypeptide, B

of
Fanconi-Bickel syndrome: G207-2 Liver/Paureas-glycogen synthesis
Coenzyme glycogen phosphorylase: BC def-hypoglycemia

decabraylate
Be dejsGABA)
seizures/nemopathy
HMP shunt
to
get s
O
mricemia
↑

Free radical scavenging:

RBC
(RLE) Lens
Reductive biosynthesis of steroids
and fatty acids:
Liver
assay for B, def. Adipose
Adrenal cortex
Gonads

Pathways without ATP:

Rossman fold: NADP binding domain - HMP slut

-
uronic acid

B x onidation/wouidation/VICFA
NADPH sources: primarily shut
-

Hop

(RBC)
Shunt - 2,3 BPG
It

rightshift
Malic
-

enzyme
-

isocitrate dissociation
->

cytoplasmic dehydrogenate ↑ On

-
seine
poisoning (glycolysiscont
ATP)
 Palmitate (16C)

cyte plasm
- e.g.
L
cat abolic
(2C
-

- insulin 8
Accyl
So A

- mitochondria -breaks
unes
↓
88 7
+
x 4 25=

glucagon 108-2 ATP

=

=
106 ATP

2 ATP used
FA SYNTHASE COMPLEX <- NADPH ATP ->
AMP

Carnitine=Lysine + methionine

productof
FA
synthesis -
stops breakdown

Malonyl CoA- Regulator

Only for >14C
Jatifsyn) can breaks down
time
sit, for long
->
down

mitochondria

reducing for
breakdown
DeH FAD H
condensing
ene

Hydratase
DeH NADH
10
ATP Thiolase
dinner
releasing Acti
coA RLE
ATP: 1.5 2.5=4 ATP +

as product
 FA oxidation and disorders
&
Jamaican
vouiting
sickness
Zellweges
Saturated FA: Boxidation Unripe Ackee fruit:Hypoglycin VLCFA+ Phytanic acid
(out 1ststep FADH Dett)
Unsaturated FA: Bonidation ↓ 1. 5 ATP Acyl CoA dehydrogenase – ACCUMULATION
Non-ketotic hypoglycemia
Odd chain FA: Acetyl
CoA+
Propionyl CoA ABSENT PEROXISOMES
B

VLCFA: Peroxisome 8C
No ATP X

CoA v
MCAD
deficiency
AcchiV
->

Have Non-ketotic hypoglycemia

Dicarboxylic aciduria oxidation w

NO ATP/ACETYLCoA: SIDS Sudden infant deali

Alpha oxidation Peronisomes, SER (Microsomes)
hypoglycemior,
no 1B y B
usually feeding
back - B Ref sum

Omega only microsomes -

dicarbonylic
acid
RP, Icthyosis, Neuropathy
-

PHYTANIC ACID RAISED

 Cholesterol synthesis fed state, insulin
cytiplasm
chol? UB
common for
Vit D, Steroid, Bile acids
HMG COA Synthase

reductase
Stations ne
a cholestent (RLE)
SIE-myopalling
:OR do est
Exc
for
.. supplement100 if
statins
taking

acids:
I' bile

↓ I
chain
cyclic
1st
acid Lillocholic acid
descrycholic
bile acids:

lives and the

ene

Entershepatic
circulation:
comebackto

(30C)
d
COB (ETC)
Lipoproteins
HD1 VIDL
chlyomicrons LDL
chol.
Max TAG Min TAG good TAG-Endogeneous Max cholesterol
Exogeneous lipid Min size
Max size Max PL
Min PL, cholesterol Max protein
Min protein Max density
Min density Fastest mobility
W

Remains at origin (alpha fraction)

W
Cholesterol metabolism
TAG
↓

glycerol + FA

Active
entre B
I
desit
e

⑪ O
-bad i Apo Al

amphipattve hydrophobic
I discoid) I spheroidal)

fat
#D1I
Reverse cholesterol transport: 3
signals:
i
E
endogenous
to ABCA1 Tangie'sdisease
ABCG1 Sitosterolemia
diet-B48
liver -B100
SRB1
IDL 2
take
E
MDL
malize -
periphery
-

fat from ID1

take it to lines

Apo

complete (CAT-
Norman's
 Urea cycle
NEWBORN WITH HYPERAMMONEMIA / HIGH GLUTAMINE, ENCEPHALOPATHY AND RESP ALKALOSIS
ornithine
to
ausposter
defective
oroticaciduria CITRULLINE B HIGH: HHH Syndrome
LOW HIGH
HOMOCITRULLINE
Megabblastic OROTIC ACID ORNITHINE
=

ARGINOSUCCINATE
anemia
I
OTC AMMONIA
↓ deficiency LOW HIGH
G
HIGH
LOW
aciduria
antic CPS+lysine homo imulline
=

CPSI XIR-OTC I
hyper ammonema II) Argino
succinate
Argino
succin axe

Hyperammonemia IS
~
1 Mc synthase
CP5-pyrimidene
syn, pathway
↓
acid
orotic

I
from Onalracetate)

g
G CPS11-pyrimidine
synthesis
·if only 1 link asked)

## Shraddhar

web's
us to
cycle
 Protein structure
bonds
peptide/covalent
most stable
-MC
- Ithanded
helin

-
Mc glycine
AA:
-
disruplei:proline

motifs

g
Not spectrophotometry

x I structure

globinaz
High-yield
URINE ODOUR allaptonia CONDITION
abo
Ferric chloride test
PRW
Guthrie test
Valine, Isoleucine, Lysine
Dinitrophenyl hydrazine test MSUD
VIL

Obermeyer test Hastump Inyptophan

- wisin
def. I, sertrain def. I

Cyanide nitroprusside test tomocystinuria

-
6,9,12

VMA Pheochromocyloma
5-HIAA Carcinoid
Syndrome

&N13
5' GATCTAC
3' DNA
assume
5'
3 C TAGATG
5'
RNA 3' UAGAUG
(T- U) C

=G

2828

A T
w
=
 Chromosomes
Chromosome- (ARBS
Lyonization
BARR BODIES
Mechanism: DNA methylation
Gene: XIST
inter life
Time: 6 days
No: XO O
XX 1
B
XY ⑧ fr
L
XXY 1 Davidson bodies
1, 2, 3 semander 13, 14, 15, noti nour
body
Buccal-Ban bodies 21,22,
XXX 2 I
neuliophil Acrocentric
lanslocation - Robertsonian
liauslocation
mech. Down's
in

suppression:knock down

miRNA/siRNA Robertsonian translocation 14,21)

20-25bp
Exportin 5 miRNA 15, 16: tumor suppressor Ring chromosome ends are lost

double sanded OncoMirs: 155, 200 Isochromosome dividing

horizontally
chromosome

hopin
cuts
small
RNA
interfering
DICER1 mutation: diXq
Mc:
loop
single
stand Pinealoblastoma MC with cancer: :17q
CRNAinduced
silencingt WT MC with testicular cancer: i22p
mRNAsequence Pleuropulmonary blastoma
Sertoli Leydig
Multinodular goiter
POLYMORPHISMS / DNA MARKERS
Repeatlenght polymorphism
SNP RLP CNV
Single
nucleotide polymoephism variations
number
STR VNTR copy
1300 bp variable
by repeated
-

short
100s
number
-

tander
-
SNP
10 million genome
in
repeats
tandem
repeats
B
15-70bp
2 -

6bp
mini satellite
microsatellite
Buffers and titration curve
Zwillier ions
⑳
proton donor

L ↓
carbonyl
~glyane
Proton- amine

acceptor
PN2

-
no ionisable side chains
Lebe 1 calia curves

PK1

x X
⑳
-
NH
-
208
High-yield

3 months

2-3 weeks
High-yield

Rolliera
d

Retone

Body

&

Benedicts Protein