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Hemoglobin Porphyrin Glycogenesis
Hemoglobin Porphyrin Glycogenesis
MITO FATO
FA oxidation
Acetyl CoA
TCA
Oxidative
Phoshorylation
Fatty Acid
Cholesterol
Synthesis
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Hemoglobin
2
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HEME
PORPHYRIN
SYNTHESIS
CYTOSOL
MITOCHONDRIA
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Uroporphyrin
Most Water soluble
porphyrin
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4
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Protoporphyrin
Least Water
soluble porphyrin
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15
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18
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Direct Bilirubin
CONJUGATED
Bilirubin
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DiRect
Hyperbilirubinemia
Dubin
Johnson
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DiRect
Hyperbilirubinemia
Rotor
Syndrome
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MRP-2
(multidrug-resistance-like
protein 2) deficiency
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GLYCOGEN
GLYCOGEN PHOSPHORYLASE
GLUCOSE 1-PHOSPHATE
PHOSPHOGLUCOMUTASE
GLUCOSE 6-PHOSPHATE
GLUCOSE
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33
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Reference
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Guru Guide
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Simple
Complex Derived
Lipids
Fats Waxes
Other
Phospholipid
Glycolipids complex
s
lipids
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LIPIDS ARE
CLASSIFIED AS
SIMPLE OR
COMPLEX
Simple lipids
b. Waxes
38
Complex Lipids
Complex lipids are esters
of fatty acids, which
always contain an alcohol
and one or more fatty
acids
Phospholipids
Glycolipids
(glycosphingolipids
Other complex
lipids: sulfolipids,
amino lipids &
Lipoproteins
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FATTY ACIDS
saturated (containing no
double bonds )
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LIPIDS
DIETARY SOURCES
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Clinical Correlation
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TABLE 21–1
Saturated
Fatty Acids
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Leukotrienes
• The leukotrienes and lipoxins
are a third group of eicosanoid
derivatives formed via the
lipoxygenase pathway
• Leukotrienes cause
bronchoconstriction
• Asthma
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17
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Plasmalogen:
Phospholipid in
mitochondria
PAF: Platelet aggregation, hypotension,
ulcer formation, inflammation
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Clinical Correlation
Loss of sphingolipids
Multiple
ethanolamine from white
Sclerosis
plasmalogen & matter
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3-phosphatidylinositol
cardiolipin (diphosphatidylglycerol)
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Membranes
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Metabolism of
phosphatidylcholi
ne (lecithin)
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Clinical Correlate
• Dipalmitoyl lecithin is a major
constituent of the surfactant
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Clinical Correlate
Phosphatidylserine also plays a role in apoptosis (programmed cell death)
60
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Sphingomyelin
s
• Sphingomyelins contain no
glycerol
• On hydrolysis à fatty
acid, phosphoric acid,
choline, and sphingosine.
• The combination of
sphingosine plus fatty acid
is known as ceramide
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Clinical
Correlate
Sphingomyelina
se
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Lysophospholipids Are
• lysophosphatidylcholine
Intermediates in the
(lysolecithin)
Metabolism of
Phosphoglycerols • promoting atherosclerosis
63
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Clinical
Correlat
e
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Lorenzo's oil is
a 4:1 mixture of
glyceryl
trioleate and
glyceryl
trierucate.
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Metachromatic Leukodystrophy
Krabbe Disease
● Arylsulfatase A ● β - galactosidase
● 3-Sulfogalactosylceramide ● Galactosylceramide
● MR, psychologic ● MR, myelin almost absent
disturbances, demyelination
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Farber Disease
Accumulation of
Ceramide
Ceramidase
Hoarseness,
dermatitis, skeletal
deformation, MR, fatal
in early life
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