1.

Outline the metabolic pathway for the synthesis degradation of glycogen

2. Describe the hexose monophosphate shunt (HMP) and its biological significance
3. Explain the complication of diabetic mellitus
4. Describe the T.C.A cycle and its regulations
5. What is the significance of glycolysis
6. Draw the cori cycle
7. Write short notes on the following
a) Lipoproteins
b) Essential fatty acids
c) Glycolipid
8. Outline the urea cycle and note the site of synthesis and what is its purpose in our body
9. Outline the B- oxidation of fatty acids
10. Write an account on the classification of lipids with suitable examples
11. List the functions proteins
12. Write short notes on phenylketonuria and Albinism
13.

Answers
1. Glycogenolysis : degradetion

Glycogen

Phosphorylase

Glucose 1 phosphate
phosphoglucomutase

Glucose 6 phosphate

phosphotase

Glucose

The pathway is influenced by the hormone called glucagon

a) Mammalian glycogen stores glucose In times of plenty (after feeding) and supplies in times of need ( during fasting, fighting and
starvation)
b) Glycogen provide fuel in the muscles for muscle constrictions and exercises
c) In the liver glycogen is converted into glucose and enters the blood stream or transported to other tissues
d) The hormones that convert glycogen to glucose are insulin, glucagon & adrenaline

Synthesis

hexokinase phosphoglucomutase UDPG pyruphosphorylase

glucokinase
Glucose G-6-P G – 1-P UDPG
ATP ADP
Glycogen synthase

Glycogen primer

glycogen

1,4- 1,6 glycosyl unit 1,4 glycosyl unit

Branched enzyme
Amylo 1,4- 1,6 tranglucosidase
 Glucose is phosphoriated to glucose 6 phosphate by an enzyme Hexokinase in the muscles and glucokinase in the liver
- Glucose 6 phosphate is converted to glucose 1 phosphate by an enzyme called phosphoglucomutase, glucose 1 phosphate reacts
with uridien triphosphate to form uridien diphosphate glucose by an enzyme called UDP-glucose pyruphosphorase.
- UDPG is transferred to pre existing glycogen molecules called glycogen primer, it links to 1,4 glycosidic linkage by the enzyme
called glycogen synthase
2. This is an alternative pathway to glycolysis and TCA cycle for the oxidation of glucose. It is located in the liver, adipose tissue, RBC,
testicles. These tissues except RBC are involved in the synthesis of fatty acids which are dependants on the supply of NADPH.

Significance of HMP shunt

- It generates pentose & NADPH
- Pentose ( ribose -5- pentose) are used in the synthesis of nucleic acid e.g. DNA, RNA and also nucleotides like ATP, NAD+ & FAD
- NADPH is required for the biosynthesis of fatty acids, steroids and glutamine acid
- The continous production of hydrogen peroxide in the living cells can chemically damage the cell and proteins but this is
prevented by the production of NADPH ( anti oxidants)
- Antioxidant drugs
- Anti biotic drugs
- Anti malaria
- Anti pyretic drugs
3.
 Retinopathy: vision impairment and blindness
Hyperglycemia leads to sobitol formation which may lead to retinol micro muscular abnormalities, leading to retinopathy
 Neuropathy: common complications
Numbness, tingling, pain, burning sensation of extremities
 Angiopathy: damage of the basement membrane of blood vessels
Increased risk of stroke and coronary artery diseases leading to myocardial malfunctions
May cause alterostrosis in media sized cerebrum leading to paralysis
 Nephropathy: damage to the glumerus of nephron of kidneys and associated capillaries this leads to decreased filtration of the
kidneys
Capillary damage is caused by Angiopathy
Urinary protein detection is useful in diagnosis of nephropathy
 Diabetic foot: foot ulcer leads to amputation
Ischemia
 Automic neuropathy: diarrhea, impotence

4. Regulation of the T.C.A cycle

It is regulated at four steps
- Pyruvate dehydrogenase
- Citrate synthase
- Isocitrate dehydrogenase
- α ketoglutarate

T.C.A cycle
 Acetyl coA is converted in citrate by an enzyme called citrate synthase
 Citrate is converted to cis-aconitate by an enzyme called aconitase
 Cis-aconitate is converted to D-isocitrate by an enzyme called aconitase
 D-isocitrate is converted to α ketoglutarate by an enzyme called isocitrate dehydrogenase
 α ketoglutarate is converted to succinyl coA by an enzyme called α ketoglutarate dehydrogenase
 succinyl coA is converted to succinate by an enzyme called succinyl coA synthase
 succinate is converted into fumarate by an enzyme called succinic dehydrogenase
 fumarate is converted to malate by ban enzyme called fumarase
 malate is converted to oxaloacetate by an enzyme called malate dehydrogenase
 significance

 it provides energy inform of ATP

 it provides substrates for respiratory chains
 it the final common pathway for the oxidation of carbohydrates, lipids and proteins
 it is both catabolic and anabolic which means amphibolic

5. – glycolysis is the principle root for glucose metabolism for the production of A.T.P molecules
- It provides pathway for the metabolism of fructose and glactose derived from a diet
 - Has the ability to provide A.T.P in the absence of oxygen and allows the tissues to survive the period of anoxic(little bit shortage of
oxygen).
- In erythrocytes, glycolysis supply 2,3 biophosphate glycerate which is required for hemoglobin functions in the transport of oxygen.

Regulations
Regulated at three steps
- Hexokinase and glucokinase
- Phosphofructokinase
- Pyruvate kinase

Glycolysis

 Glucose is converted to glucose 6 phosphate by an enzyme called Hexokinase

 Glucose 6 phosphate is converted to fructose 6 phosphate by an enzyme called Phosphoglucose isomerase
 Fructose 6 phosphate is converted to fructose 1, 6 biphosphate by an enzyme called Phosphofructokinase
 Fructose 1, 6 biophosphate is converted to dehydroxyacetone phosphate by an enzyme called aldolase
 Dehydroxyacetone is converted to glycealdehyde 3 phosphate by an enzyme called triose phosphate isomerase
 Glyceraldehyde is converted to 1,3 biphoshogycerate by an enzyme called glyceraldehydes phosphate dehydrogenase
 1,3 biphosphate is converted to 3 phosphoglycerate by an enzyme called phosphoglycerate kinase
 3 phosphoglycerate is converted to 2 phosphoglycerate by an enzyme called phosphoglycerate mutase
 2 phosphoglycerate is converted to phosphoenol Pyruvate by an enzyme called enolase
 Phosphoenol Pyruvate is converted to Pyruvate by an enzyme called Pyruvate kinase

6. Cori cycle

Glucose (glycogen) is converted to lactate in the muscles and lactate is converted back to glucose in the liver.
Liver blood muscle
Glycogen
glycogenesis

glucose glucose glucose

Glucose 6 phosphate anaerobic glycolysis

gluconeogenesis

pyruvate lactate lactate

7. Lipoprotein: since lipids are water insoluble they are present in the blood in form of lipoproteins which are water soluble. They are also
surrounded by a shell called apoprotein phospholipids and free cholesterol. They have an outer polar shell which makes them soluble in
water. There are different types of lipoproteins….
- High density lipoprotein (HDL)
- Low density lipoprotein (LDL)
- Very low density lipoprotein (VLDL)
- Chylomicrons

Glycolipids: they contain fatty acids, spingosine (alcohol, carbohydrates) or carbohydrates derivatives e.g. Cerebroside is present in the
white matter of the brain and ganglioside (found in the grey matter of the brain)

Essential fatty acids: these are fatty acids which are not synthesized in the body but are supplied through diet
- They contain more than one double bond e.g. linoleic, linolenic, archidonic acid
- These fatty acids are not synthesized in the human body because they lack desaturase enzyme which introduces double bonds
beyond 9th 10th

Diabetics type 1
- Caused by lack of insulin secretion due to distractions of pancreatic cells bita cells
- Viral infections
- Auto immune disorders
- They may be hereditary tendency bita cell degeneration
- This occurs between the age of 12 & 15 years
 Symptoms
- Urinating frequently ( polyuria)
- Excessive thirst (polydipisia)
- Excessive hunger ( polyphasia)
- This patient may not be obese
Type 2
- This is adult onset
- Decreased sensitivity or decreased response to insulin
- Inadequate insulin receptors
- Obesity is a common cause

8. Proteins play a very central role in cell functions and cell structure. It constitutes 17% of body weight
- Proteins form an essential part of a particular structure e.g. membrane, muscles, connective tissues and organs e.g. liver, heart
- Various proteins are enzymes in nature and catalyze biological reactions.
- Several proteins acts as hormones and thus regulate various metabolic processes of the body e.g. insulin
- A number of proteins serve as carriers of transport of various substances e.g. hemoglobin
- Some proteins acts as receptor molecules for the transport of compounds across the cell membrane such as common receptors
- Various proteins bind to certain substances and store them in different tissues, acting as storage protein ferritin
- Some proteins like gamma(y) globulin acts as anti bodies and provide immunity
- Proteins acts as buffers to maintain the PH of the cell e.g. plasma proteins & HB
9. Phenylketonuria is caused by defect in gene producing a liver enzyme. It is an inborn error of phenylalanine metabolism associated within
an inability to convert phenylalanine into tyrosine
- There is accumulation of phenylalanine in body fluids
- Failure to convert phenylalanine to tyrosine results in accumulation of toxic derivatives such as phenyl Pyruvate, phenyl acetate,
phenyl lactate, phenyl acetyl glutamic acid.
- Almost all untreated PKU patients are severely mentally retarded.
PKU is divided in 3 broad groups caused by the deficiency of phenylalanine hydroxylase

Characteristics of PKU
A. Increased level of phenylalanine, phenyl lactate, phenyl acetate, phenyl Pyruvate and phenyl acetyl glutamine in tissue, plasma and urine
B. Neurological symptoms
- Mental retardation
- Failure to walk
- Failure to talk
- Seizures
- Trauma
- Failure to grow

C. Hypo pigmentation: PKU patients have a lighter skin color, fair hair and blue eyes due to deficiency of pigment melanin.
Treatment: the therapy of PKU is low phenylalanine diet. The aim is to meet the needs for growth and replacement. Low phenylalanine
diet should be start very soon after birth to prevent irreversible brain damage.

ALBINISM: is a defect tyrosinase. Affected individuals cannot synthesize melanin.

Biosynthesis of melanin pigment

- The synthesis of melanin occurs only in pigment producing cells called melanocytes

Tyrosine

tyrosinase

DOPA

DOPA quinine

Melanin
Symptoms
- Photophobia
- White hair and skin
 - Nystagmus
10. There are basically two types of glycosuria : hyperglycemic glycosuria: alimentary, nervous or emotional, glycosuria due to endocrine
disorders & renal glycosuria acquired, hereditary, experimental glycosuria
11. B – oxidation is the process by which fatty acids in form of Acyl coA are broken down in the mitochondria and/ or perixsomes to generate
Acetyl coA then entry molecule of T.C.A

Acyl coA

Acyl coA dehydrogenase

1. Dehydrogenation Trans Δ- 2 Enoyl coA

Enoyl coA hydrotase

2. Hydration L – 3- hydroxyl coA

L -3-hydoxyl coA dehydrogenase

3. Dehydrogenation 3 ketoacyl coA

thiolase

4. Thiolytic cleavage Acetyl coA, Acetyl coA

Acetyl coA

T.C.A