3/25/23

MITO FATO
FA oxidation
Acetyl CoA
TCA
Oxidative
Phoshorylation

Give BOTH of you

a HUG
H eme
Urea
Gluconeogenesis

Fatty Acid
Cholesterol
Synthesis

1
 3/25/23

Hemoglobin

2
 3/25/23

HEME
PORPHYRIN
SYNTHESIS
CYTOSOL
MITOCHONDRIA
7

3
 3/25/23

10

11

Uroporphyrin
Most Water soluble
porphyrin

12

4
 3/25/23

Protoporphyrin
Least Water
soluble porphyrin

13

14

15

5
 3/25/23

16

17

18

6
 3/25/23

19

Direct Bilirubin
CONJUGATED
Bilirubin

20

DiRect
Hyperbilirubinemia
Dubin
Johnson
21

7
 3/25/23

DiRect
Hyperbilirubinemia
Rotor
Syndrome
22

MRP-2
(multidrug-resistance-like
protein 2) deficiency

23

24

8
 3/25/23

25

26

27

9
 3/25/23

28

29

30

10
 3/25/23

What enzyme will conjugate

unconjugated bilirubin?
Uridine Glucuronyl
Transferase

31

GLYCOGEN
GLYCOGEN PHOSPHORYLASE

GLUCOSE 1-PHOSPHATE
PHOSPHOGLUCOMUTASE

GLUCOSE 6-PHOSPHATE

GLUCOSE

32

33

11
 3/25/23

34

Reference

35

Guru Guide

36

12
 3/25/23

LIPIDS ARE CLASSIFIED

AS SIMPLE OR
COMPLEX
LIPIDS

Simple
Complex Derived
Lipids
Fats Waxes
Other
Phospholipid
Glycolipids complex
s
lipids

37

LIPIDS ARE
CLASSIFIED AS
SIMPLE OR
COMPLEX

Simple lipids

a. Fats and Oils

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

39

13
 3/25/23

Fatty acids that occur in

natural fats usually contain an
UNSATURATED
SATURATED &

FATTY ACIDS

even number of carbon atoms.

saturated (containing no
double bonds )

unsaturated (containing one or

more double bonds )

40

LIPIDS
DIETARY SOURCES

NATURAL DIETARY FATS

• Triglycerides (fatty acids) - 98 -
99%
• Monoglycerides and diglycerides
• Phospholipids and sterols

41

Clinical Correlation

Trans Fatty Acids NSAIDS

● Competes with EFA ● Cyclooxygenase inhibitor
● Promotes ● COX 1 inhibition –
hypercholesterolemia & stomach irritation
atherosclerosis

42

14
 3/25/23

43

TABLE 21–1
Saturated
Fatty Acids

44

TABLE 21–2 Unsaturated Fatty Acids of Physiologic and Nutritional Significance

45

15
 3/25/23

TABLE 21–2 Unsaturated Fatty Acids of Physiologic and

Nutritional Significance

46

TABLE 21–2 Unsaturated Fatty Acids of Physiologic and

Nutritional Significance

47

48

16
 3/25/23

Leukotrienes
• The leukotrienes and lipoxins
are a third group of eicosanoid
derivatives formed via the
lipoxygenase pathway

• Leukotrienes cause
bronchoconstriction

• Potent proinflammatory agents

• Asthma

49

ω3 Fatty Acids Are Anti-

Inflammatory and Have
Health Benefits

Long-chain ω3 eicosapentaenoic docosahexaenoic Anti-inflammatory

α-linolenic (ALA) (EPA) (DHA)
fatty acids effects

50

51

17
 3/25/23

Plasmalogen:
Phospholipid in
mitochondria
PAF: Platelet aggregation, hypotension,
ulcer formation, inflammation

52

• These compounds constitute as much as 10 to 30%

of the phospholipids of brain and heart.
Plasmalogens Occur
in Brain & Muscle • Protective effect against reactive oxygen species

53

Clinical Correlation

Loss of sphingolipids
Multiple
ethanolamine from white
Sclerosis
plasmalogen & matter

54

18
 3/25/23

phosphatidic acid is substituted by the substituents shown to form the

phospholipids:
(A) 3-phosphatidylcholine, (B) 3-phosphatidylethanolamine, (C) 3-
phosphatidylserine

55

3-phosphatidylinositol
cardiolipin (diphosphatidylglycerol)

56

• Glycerophospholipids containing choline,

(phosphatidylcholines, commonly called lecithins) are the
Phosphatidylcholines most abundant phospholipids of the cell membrane and
(Lecithins) and represent a large proportion of the body’s store of choline.

Sphingomyelins Are • Choline is important in nervous transmission, as

Abundant in Cell acetylcholine, and as a store of labile methyl groups.

Membranes

57

19
 3/25/23

Metabolism of
phosphatidylcholi
ne (lecithin)

58

Clinical Correlate
• Dipalmitoyl lecithin is a major
constituent of the surfactant

• preventing adherence, due to surface

tension, of the inner surfaces of the
lungs.

• respiratory distress syndrome.

59

Clinical Correlate
Phosphatidylserine also plays a role in apoptosis (programmed cell death)

60

20
 3/25/23

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

61

Clinical
Correlate

Sphingomyelina
se

62

Lysophospholipids Are
• lysophosphatidylcholine
Intermediates in the
(lysolecithin)
Metabolism of
Phosphoglycerols • promoting atherosclerosis

63

21
 3/25/23

Clinical
Correlat
e

64

65

• GM1 , a more complex ganglioside derived from

GM3, is of considerable biologic interest, as it is
known to be the receptor in human intestine for
cholera toxin

66

22
 3/25/23

Lorenzo's oil is
a 4:1 mixture of
glyceryl
trioleate and
glyceryl
trierucate.

67

Lipid Storage Diseases (Sphingolipidoses)

Tay-Sachs Disease Fabry Disease

● Hexosaminidase A ● α - galactosidase A
● Ganglioside ● Globotriaosylceramide
● MR, blindness, muscular ● Skin rash, kidney failure
weakness

68

Lipid Storage Diseases (Sphingolipidoses)

Metachromatic Leukodystrophy
● Arylsulfatase A
● 3-Sulfogalactosylceramide
● MR, psychologic
disturbances, demyelination
Krabbe Disease
● β - galactosidase
● Galactosylceramide
● MR, myelin almost absent

69

23
 3/25/23

Lipid Storage Diseases (Sphingolipidoses)

Gaucher Disease Niemann-Pick Disease

● β - glucosidase ● Sphingomyelinase
● Glucosylceramide ● Sphingomyelin
● Hepatosplenomegaly, long ● Hepatosplenomegaly, MR,
bone erosion, MR fatal in early life

70

Lipid Storage Diseases (Sphingolipidoses)

Farber Disease
Accumulation of
Ceramide

Ceramidase
Hoarseness,
dermatitis, skeletal
deformation, MR, fatal
in early life

71

24