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MOVIE
Acetyl-CoA Carboxylase reaction
Acetyl-CoA Carboxylase reaction
NAD
+
E-FADH2 Fe3+ Fe2+ Stearoyl-CoA + O2
-O
O stearate
-O
O oleate
Double bonds are introduced into long-chain acyl-CoAs through an
electron-transfer process coupled to the reduction of molecular
oxygen
Reaction catalyzed by a complex of membrane-bound enzymes
Double bonds inserted such that the new double bond is three
carbons closer to the CoA group, and never beyond the C9 position
ESSENTIAL FATTY ACID
O O O
O O O
O R1 O R1 O R1
+
R2 O H O R2 O H O R2 O H O NH3
O P O + O P O O-
O P O +
NH3 N(CH3)3
O- O- O-
O
phosphatidylethanolamine (PE) phosphatidylcholine (PC) phosphatidylserine (PS)
Lesitin O
O O O
O
O O R1
O R1 O O
O R1 O R1 R2 O
R2 O H O R2 O H O O
OH OH R2 O H O
O P O O P OCH2CHCH2O P O
O P O
phosphatidyl- O- HO phosphatidyl- O- CHOH O- OH O-
inositol (PI) OH glycerol (PG)
CH2OH cardiolipin (CL)
OH
serine
CMP
O
O
O R1
+
R2 O H O NH3
O P O O-
O-
O
phosphatidyl-
serine (PS)
CTP: Citidene Tryphosphate
Biosynthesis Of Phospatidylcholine:
CDP-Choline & CDP-ethanolamine
NH2
N O
O O
ATP ADP O CTP PPi
+ O P O P O
HO + -O P O + (H3C)3N O
N(CH3)3 N(CH3)3 O- O- H H
O-
CDP-choline H H
choline phosphocholine OH H
1,2 diacylglycerol
CMP
O
O
O R1
R2 O H O
O P O +
N(CH3)3
O-
phosphatidlycholine (PC, lecithin)
PHOSPHOLIPID SYNTHESIS
03/01/17 36
KETONE BODIES
1. Initial condensation
2. Ester condensation to
form HMG-CoA (also
precursor in cholesterol
biosynthesis)
3. Acetoacetate and acetyl-
CoA formed in a
mechanism similar to the
reverse of the citrate
synthase reaction
Metabolic Conversion of Ketone Bodies to Acetyl-CoA S
Acetoacetate reduced to
hydroxybutyrate in an
NADH-dependent reaction
Acetoacetyl-CoA can be
cleaved by thiolase to give
2 acyl-CoA
The liver can supply
acetoacetate to other
tissues
SPHINGOLIPIDS
OH (CH2)12CH3
O
R2 N H
H
O X
sphingolipid
X=H ceramide
X = carbohydrate glycosphingolipid
X = phosphate ester sphingophospholipid
OH (CH2)12CH3
O
R2 N H O
H +
O P O
N(CH3)3
O-
sphingomyelin
SPHINGOLIPIDS
SPHINGOLIPIDS
ceramide
Gal Glc
ceramide
NAN
GalNAc Gal Glc
ganglioside GM3
NAN
+
ganglioside GM2
N-acetyl
Gangliosides are degraded inside galactosamine
lysosomes by the sequential removal of
terminal sugars
In Tay-Sachs disease, ganglioside GM2
accumulates because hexosaminidase
activity is absent
This ganglioside interferes with neuronal
function
Genetic recessive disease
MEMBRANE LIPIDS