CARBOHYDRATES

Definition
• Polyhydroxy aldehydes or ketones Or produce
them on hydrolysis.
• Water soluble in Nature
• 1 gm of carbohydrate Yield 4.5 Kcal of energy
• Can be stored in body
• Performs various functions
 Classification
1. Monosaccharides
a. Aldoses
b. Ketoses
2. Oligosaccharides
a.Diasaccharides
b.Trisaccharides
c.Tetrasaccharides
 Monosaccharides
No. of carbons Aldose (- CHO) Ketose (-C=O)
3c Glyceraldehyde 3P DHAP
4c Erythrose 4 P Erythrulose 4P
5c Ribose 5P Ribulose 5P
6c Glucose 6P Fructose 6P
 Derivatives of Monosaccharides
1. Sugar acids
2. Sugar alcohols
3. Osazones
4. Phosphoric acid esters
5. Amino sugars
 Disaccharides: Biomedical importance
Name Linkage Importance
Maltose Formed in GIT on digestion of starch
(α1 -> 4)
(Glu+Glu) and glycogen by amylase
Lactose (β1 -> 4)
(Gal+glu)
Sugar present in milk
Sucrose (α1 -> β2)
(Glu+fru)
Table sugar

Isomaltose Formed in GIT on digestion of starch

(α1 -> 6)
(Glu+glu) and glycogen by amylase
Trehalose
(Glu+glu)
(α1 -> 1) Present in the diet
 Polysaccharides
Homopolysaccharides
1. Starch (α14 and α16)
2. Glycogen(α14 and α16)
3. Dextrins (α14 and α16)
4. Dextran (α14 ,α16 and α13)
5. Cellulose (β14)
6. Chitin (Nac Giusamine, β14)
7. Inulin( Fructosan)
8. Agar(Galactasan)
 Heteropolysaccharides
Repeating
Name Occurrence
Disaccharides
Hylaluronic acid D-Glucuronic acid-N-
Joints, Ocular fluids
acetyl glucosamine
D-Glucuronic acid-N-
Chondroitin sulphate Cartilage, Tendons,
acetyl galactosamine
Bone
4 S/6S

Glucoronic acid –N-S-

Heparin Mast cells, liver
glucosamine
 Heteropolysaccharides
Name Repeating Occurrence

Disaccharides
Dermatan Iduronic acid-N- Skin,valves,
sulphate acetyl blood vessels
galactosamine 4S
Keratan Sulphate D- Galactose-N- Cartilage and
S- glucosamine Cornea
 Glycoproteins/Proteoglycans
• Extracellular Macromolecules
• Depending on their carbohydrate and protein content they
are classified as--
• 1.Glycoproteins-Carbo< proteins
• -Oligosaccha.(1-20), branched
• Eg- Igs, Transferrin ets…
• 2.Proteoglycans-Carbo> proteins
• -Longer(100), Linear, Unbranched
• Eg.- Decorin, Perlecan ,aggregan
 Glycoproteins
• There are 2 types of glycoproteins
• 1.O-linked
• 2.N-linked
• Functions of glycoprotein
1. Lubrication
2. Cell adhesion
3. Cell recognition
4. Sugars in Glycoprotein stabilizes protein against
degradation, protect from proteolysis
 Structure of glycoproteins
• Polypeptide chains of proteins aa xyl
covalently linked to oligosaccharide
chains of carbohydrate
aa gal man gal
• There maybe 1-30 or even more
oligosaccharide chains
• The no. of mono saccharide residues aa
may vary from 1-2 to much larger
• The mono saccharide residues are
mainly xylose, fucose, gal, man, gluN, aa
galN, manN, NANA
 Glycoprotein degradation
• Lysosomal enzymes glycosidases
1. Exoglycosidases
2. Endoglycosidases
Genetic deficiency of these enzymes results in partially
degraded glycoproteins and oligosaccharides in cell.
These disorders are termed as glycoprotein storage
diseases.
 Proteoglycans
Functions
1.Polyionic
2.Highly hydrophilic
3. Forms gel like substance acts
as lubricants and cushions
4.Provides rigidity and stability to
connective tissue
5.Mediate cell -cell interactions
 Mucopolysaccharidosis
Accumulated
Type Syndrome Enzymatic Defect
Meta.
I Hurrler’s Alpha-L-Iduronidase DS,HS
II Hunter’s Iduranate Sulphatase DS,HS
III Sanfilippo’s Heparin Sulphasate HS
Galactosaminesulphat
IV Morquio’s KS,CS
ase
 Accumulated
Type Syndrome Enzymatic Defect
Meta.

V Scheie’s L-Iduronidase DS

N-
Maroteaux-
Vi Acetylgalactosamin DS
Lamy’s
e-4-sulphatase

vII Sly’s Beta-Glucoronidase DS,HS

 Digestion and absorption
Starts in mouth: Salivary amylase
Stomach: No significant digestion
Small Intestine: Sucrase,Lactase,Maltase,Isomaltase,
pancreatic Amylase
Digestive products: Glu,Gal,Fru,
Absorption :GLUTS (1-7)
Mode of absorption: facilited transport and Sec. AT
 Glycolysis
• Only pathway which can operate in both the
conditions:
1. Aerobic ( In presence of Oxygen)
Glucose ------> Pyruvate
2. Anaerobic(In absence of Oxygen)
Glucose ------> Lactate
 Glycolysis
1. Location: Most of the mammalian cell
2. Site: Cytosol
3. Precursor: Glucose
4. End product: Pyruvate/Lactate
5. Significance: Energy ( ATPs)
6. Regulatory Enzyme: a. GK/HK
b. PFK
c. PK
 Energetics of glycolysis
Enzyme Type of No. of ATP produce
phosphorylation
Glucokinase/Hexokinase - -1
Phosphofructokinase - -1
Glyceraldehyde 3P Oxidative 3 + 3=6 or(5)
dehydrogenase
Phosphoglycerate Kinase Substrate Level 1 + 1=2
Pyruvate Kinase Oxdative 1 + 1= 2
Total 10-2= 8 or 7
 Link between Glycolysis and TCA
• Pyruvate dehydrogenase complex (PDH)
• Multienzyme complex
• Requires 5 coenzymes
• NAD+
• TPP
• Lipoic acid
• CoA
• FAD
 TCA/Citric Acid Cycle/Kreb’s Cycle
1. Location: Cells containing Mitochondria
2. Site: Mitochondrial Matrix
3. Precursor: Acetyl CoA
4. End product: ATPs+ CO2+H2O
5. Significance: Energy ( ATPs)
6. Regulatory Enzyme: a. Citrate Synthase
b. Isocitrate DHGNS
c. α-KG DHGNS
d. Succinate- thiokinase
 TCA/Citric Acid Cycle/Kreb’s Cycle
• Special features:
1. Anabolic + Catabolic role = Amphibolic
2. Produces Plenty of energy
3. Links various metabolic pathways
4. Plays Important role in metabolic switch over
eg. Extra glucose is converted to Fat
 Energetics of TCA
Enzymes Phosphorylation No. of ATPs prod
Isocitrate dehydrogenase Oxidative 3 (2.5)
α-KG dehydrogenase Oxidative 3 (2.5)
Succinate Thiokinase Substrate level 1
Succinate dehydrogenase Oxidative 2 (1.5)
Malate dehydrogenase Oxidative 3 (2.5)
Total 12 or 10
 Gluconeogenesis
1. Location: Mainly in liver and kidneys
2. Site: Mitochondrial Matrix and cytosol
3. Precursor: Lactate,pyruvate,isocitrate,α-KG,Succinyl
CoA,Glycerol,Odd chain Fas,glucogenic AA
4. End product: Glucose
5. Significance: Synthesizes glucose from non carbohydrates sources
6. Regulatory Enzyme: a. PC
b. PEP-CK
c. Fru 1,6 BPtase
d. Glucose 6 Ptase
 HMP shunt
1. Location: Adrenal cortex,lactating mammary
glands, gonads
2. Site: Cytosol
3. Precursor: Glucose
4. End product: NADPH and fru 6 P, Gly 3P
5. Significance: NADPH ,Ribose5P
6. Regulatory Enzyme: a. Glucose 6P DHGNS
b. 6 P gluconate DHGNS
 Significance of HMP shunt
1. Provides two important intermediates
a. NADPH + H+
- Required for synthetic pathways like FA
synthesis, Cholesterol synthesis
b. Ribose 5P
- Pentose sugar present DNA,RNAs and All
Nucleotides
 Disorders of HMP shunt
• 1.Glucose 6 phosphatase deficiency
• -No NADPH No reduction of
glutathioneDrug induced Oxidative stress
can not be metabolised leads to hemolytic
anaemia.
• 2.Wernicke- Korsacoff syndrome
• -TPP deficiency especially in alcoholics
 Uronic acid pathway
1. Location: Adrenal cortex,
2. Site: Cytosol
3. Precursor: Glucose
4. End product:
5. Significance: Provides Glucuronic acid,
Ascorbic acid
 Glycogen
Metabolism
 Glycogen metabolism
Introduction
• Glycogen metabolism involves 2 pathways
– Glycogenesis: synthesis of glycogen from glucose
– Glycogenolysis: breakdown of glycogen to form glucose
• Pathways occur in the liver and skeletal muscle
• The protein on which the glycogen molecule is assembled
is called glycogenin. This protein is also an enzyme and
catalyzes the addition of first few molecules of glucose on
itself
Management
• In disorders leading to hypoglycemia
administration of uncooked cornstarch or
nasogastric feeding between meals is
required
• In disorders leading to muscle fatigue
fructose infusion during exercise is the
preferred treatment.
 Disorders of Galactose Metabolism
Galactosemia
1. Galctokinase
2. Galactose 1 P uridyltransferase (classical) -
Galactosemia,Galactosuria,Hypoglycemia,Acc of Gal
1PDecreased PiLiver Dysfunction
Glycogen storage in liver, Galactitol/dulcitol-
Cataract in earlyage.Hemolysis(lowNADPH)
3. Epimerase (Rare)
 Disorders of Fructose Metabolism
1. Essential fructosuria (Rare)
-Fructokinase is deficient
- High plasma fructose
-Fructosuria
- Asymptomatic

1. Hereditary fructose intolerance

a. Aldolase B
b. fructose 1,6 Bi phosphatase
 Diabetes mellitus- metabolic rearrangement
• Insulin Deficiency-decreased glucose uptake
• Decreased of Glycolysis
• Increased of gluconeogenesis
• Increased glycogenolysis
• Decreased glycogenesis
• Increased Lipolysis
• Decreased lipogenesis
• Increased proteolysis
 Diabetes mellitus- other consequences
1. Glycosylation of proteins. eg-
Hemoglobin, albumin
2. Sorbitol pathway: Accumulation of
sorbitol leads to Cataract, nephropathy
and peripheral neuropathy.
 Lipids
• Organic, insoluble molecules which are
soluble in organic solvents.They are
esters of Fatty acids with alcohol and
actually or potentially related to fatty
acids.
 Classification
1. Simple lipids: Fats /oils (TAG)and Waxes
2. Compound lipids: PL,GL,SL,LP
3. Derived lipids: FA,Alcohols,Cholesterol
 Digestion and absorption of lipids
• Mouth: Lingual lipase
• Stomach :Gastric lipase
• Small intestine: Pancriatic Lipase
Phospholipase
Cholesterol esterase
Emulsification
Micell formation
Adsorption of FFA, Glycerol,LysoPL,Chole
Resynthesis of LipidsPacked in Chylomicrones
 Lipid transportation
• Lipoproteins
Chylomicrons- Apo B48,TAG,Chole-
E(InstLiver)
VLDL-ApoB1oo,TAG,Chole-E (LiverPeriphery)
IDL-ApoB100,Chole-E (LiverPeriphery)
LDL-ApoB100,Chole-E (LiverPeriphery)
HDL- ApoA, Chole-E(Periphery Liver)
 Beta oxidation of Fatty acids
1. Location: Peripheral tissue
2. Site: Mitochondrial matrix
3. Precursor: Acyl CoA
4. End product: Acetyl CoA
5. Significance: Energy ATPs
6. Regulatory Enzyme: carnitine acyl coA
transferase I
 Energetics
Enzyme/ Reaction ATPs
Acyl CoA FADH2 2 x7=14 or 1.5x7=10.5
dehydrogenase
Enoyl CoA NADH+H+ 3x7=21 or 2.5x7=17.5
Dehydrogenase
Acetyl CoA TCA 12x8=96 10x8=80
Oxdaition
Total =131-2=129 or
=108-2=106
 FA synthesis
1. Location:Liver,Lactating mammary glands,Adipose
tissue,Kidneys,Brain
2. Site: Cytosol
3. Precursor: Acetyl CoA
4. End product: Acyl moeity(Palmitate)
5. Significance: Fatty acid-Required for synthesis of
other compounds
6. Regulatory Enzyme: acetyl CoA carboxylase
 Chain Elongation
• Two types
1. Microsomal
2. Macrosomal
3. Endoplasmic Reticulum (Desaturases)
 Cholesterol biosynthesis
1. Location:Liver,adrenalcortex,Intestine,ovaries,
Skin,Arterial wall etc.
1. Site: Cytosol and ER
2. Precursor: Acetyl CoA
3. End product: Cholesterol
4. Significance: Cholesterol-Required for synthesis of
other compounds
5. Regulatory Enzyme: HMG-CoA Reductase
 lipoproteins
1. Chylomicrons
2. VLDL
3. IDL
4. LDL
5. HDL
 Types of hyperlipoproteinemias
Type Lipoprotein fraction elevated
1 Chylomicrons
2a LDL
2b LDL+VLDL
3 Chylomicron remnants and IDL
4 VLDL
5 VLDL + Chylomicrons
 Prostaglandins
• Derivatives of arachidonic acid commonly known
as eicosanoids.
• Local Hormones-Autocrine, Paracrine
• Inflammatory mediators(Fever)
• Vasoconstrictor
• Vasodialators
• Uterine contraction