Mission NEET PG Biochemistry Day - 4 by DR Smily Pruthi

Mission NEETPG
“Your Course Completion, Our Responsibility"
Day 4
Lipids & Vitamins
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Mission NEETPG
• Definition : compound which is insoluble in water
• Soluble in non polar organic solvent
• Fatty acid
• Fat
nick name of fatty acid – ‘Acyl’

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Lipids
Simple Lipids Complex Lipids

(Fatty acid + Alcohol) (Fatty acid + Alcohol + other component)
Phosphate
Carbohydrate
Phosho- Glyco-
Lipids Lipids

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Simple Lipids

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Phospholipids
alcohol + fatty acid + phosphate

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Phospholipases

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Cardiolipin
• Used in VDRL test

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Anti-phospholipid syndrome

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 Anti-phospholipid syndrome patients

give false positive VDRL test, as they
have antibodies against cardiolipin &
VDRL test reagent has Cardiolipin
antigen.

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• Phospholipids are of 2 types:

1. Glycero-phospholipids
2. Sphingo- phospholipids

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Sphingo- phospholipids
• Alcohol + fatty acid + phosphate

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Glycolipids
• Sphingosine + FA + glu→ glucosyl ceramide
• Sphingosine + FA + gal → galactosyl ceramide

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Sphingolipidoses

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Other sphingolipidoses
• GM1 Gangliosidosis – beta-galactosidase
• Metachromatic Leukodystrophy – cerebroside sulfatase

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• All are Autosomal recessive , Except Fabry’s disease – XR

• All have mental retardation, Except : Gaucher’s disease
• All have hepatosplenomegaly, Except : Tay Sach’s disease,
Krabbe’s disease
• All have cherry red spots EXCEPT: Fabry’s , Gaucher’s,
Krabbe’s

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Gaucher’s disease
• Most common lysosomal storage disease
• ERT (enzyme replacement therapy) available
• Bony pain, pathological fracture, easy bruisability
Macrophages:
• Nucleus – eccentric
• Cytoplasm – crumpled tissue paper appearance or wrinkled
appearance

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Gaucher cell

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Treatment in Gaucher’s
• Acid glucosidase → Imiglucerase, Velaglucerase alpha , Taliglucerase
alpha

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• Rice water diarrhoea- Cholera-
intoxication is due to which
ganglioside: GM-1 ganglioside
receptor
• Cholera toxin binds oligosaccharide
portion of GM1 ganglioside
• One here means one sialic acid
• Tetanus and botulinum toxins

selectively bind gangliosides of
the G1b series, namely, GT1b,
GD1b, and GQ1b
•

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Wolman’s disease
• Lysosomal storage disease
• Enzyme – lysosomal acid lipase
• Watery green diarrhoea
• Vomiting, failure to thrive
• Hepatosplenomegaly
• Calcification of adrenals – pathognomic feature

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Q. Which of the following is not a product of complete hydrolysis of Sphingomyelin?
a. Choline
b. Phosphate
c. Ceramide
d. Palmitic acid

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Lipotropic
Factors

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Lipotropic Factors
• PUFAs
• Vitamin – B9 & B 12
• Amino acids → Glycine, Serine, Methionine
• Choline
• Betaine
• Inositol

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PUFAs
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• Many patients of Retinitis pigmentosa have decreased DHA levels (NEET JAN 19 )
• DHA is found in photoreceptor cells.
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Q. Which of the following processes yields arachidonic acid in mammals?
a) Elongation of stearic acid
b) Chain elongation and one desaturation of linolenic acid
c) Chain elongation and two desaturations of linoleic acid
d)Chain elongation and one desaturations of linoleic acid

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Q. C17 H31 COOH is formula of:
a. Linoleic acid
b. Linolenic acid
c. Arachidonic acid
d. Timnodonic acid

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Lipoproteins

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Lipoprotein Another name

VLDL Pre-beta
IDL Broad beta
LDL Beta
HDL Alpha

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NEET 2019
• Apolipoprotein responsible for Alzheimer dis → Apo E4

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Tangier’s disease/ Familial alpha-lipoprotein

deficiency/
Hypo alpha lipoproteinemia:
• Mutation -ABC-A1 transmembrane transport protein
which helps in efflux of cholesterol & PL from
peripheral tissue to HDL.
• Cholesterol accumulation
• Like in tonsils→ Large orange/yellow tonsils →
characteristic feature
• Enlargement of throat, Liver, Spleen
(hepatosplenomegaly), Lymph Nodes
• Peripheral Neuropathy (Mononeuritis multiplex) +

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Fatty acid synthesis

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Cytoplasm
Citrate activates Acetyl CoA carboxylase

53
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Ketone body pathways

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• Starting point of ketone body synthesis -
• First enzyme of ketone body synthesis -
• First enzyme of ketone body utilisation -
• Immediate precursor of acetoacetate -

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Beta-oxidation
of
Fatty acids

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MCAD deficiency

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Ketotic Hypoglycemia Non-Ketotic Hypoglycemia
Von Gierke’s disease Insulinoma

Alcoholism Defect in beta oxidation (MCAD
deficiency, Jamaican vomiting
sickness, Carnitine or CPT-1 or
CPT-2 deficiency )
Starvation

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Refsum’s dis
• Enz- phytanic acid alpha oxidase/ phytanoyl CoA alpha hydroxylase
• C/F – cardiac arrythmias, retinitis pigmentosa , peripheral neuropathy
• Anosmia, deafness, ichthyosis, ataxia
• Heart, eyes, CNS affected

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Vitamin Common Specific

deficiency
B6
B9
B-12

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Cholesterol Synthesis

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Acetyl CoA Acetyl CoA (2C)
Acetoacetyl CoA Acetoacetyl CoA (4C)
HMG CoA HMG CoA (6C)

HMG CoA Reductase(RLE)
Acetoacetate Mevalonate (6C)
Farnesyl PPi (15 C) Synthesis

of CoQ
Squalene (30 C)
Ketone body
Lanosterol (30 C)
synthesis
in
Cholesterol (27 C)
mitochondria
Cholesterol synthesis in cytoplasm
69
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• Lanosterol is the first steroid or first cyclic compound formed in chol

synth

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Vitamins & Miscellaneous

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Atypical Vitamins

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• B1
• B6
• B7

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Gingival bleeding
Perifollicular haemorrhages
Glossitis/ Majenta tongue

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Photosensitive dermatitis “Your Course Completion, Our Responsibility"
Casal’s necklace

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Follicular hyperkeratosis

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Sign of Vitamin A deficiency Sign of Vitamin A toxicity

• Xerophthalmia • Raised Intracranial tension
• Keratomalacia resembling Brain Tumour
• Immunosuppression (Known as Pseudo Tumour
• Skin ulceration Cerebri)
• Corneal ulceration, • Hepatomegaly
scarring • Liver damage
• Atrophy of urinary & • Hyperlipidemia
respiratory tract • Blurred Vision
epithelium leading to • Alopecia
infection • Dry pruritic skin

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Most sensitive screening for Vitamin A

deficiency- Plamsa retinol (main circulating form
of Vitamin A).
This can assess subclinical vitamin A deficiency

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HEPCIDIN

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Menke’s Wilson’s
• XR • AR
• Cu deficiency • Cu excess
• ATP-7A protein • ATP-7B protein
• Present in intestine for • Present in liver to throw Cu in
absorption of Cu bile & incorporate Cu in Cp
Ceruloplasmin (Cp) decreased in both

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Menke’s disease
Wilson’s disease
Kayser-Fleischer ring
(Descemet’s membrane of
cornea)
Sunflower cataract
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Menke’s disease
premature birth
hypotonia
growth retardation
mental retardation
grey –depigmented hair (tyrosinase
affected)
brittle kinky hair (collagen affected)
decreased Cu in blood & urine

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Wilson’s
Cu increased in :
• Liver – damage
• Brain – neurological degeneration
• Kidneys – Renal damage, urolithiasis
• Bone marrow & RBCs – hemolytic anemia

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Nutritional deficiencies
Kwashiorkor Marasmus
• In preschool children (1-5 years) • In infants (<1 year)
• Due to low protein intake • Due to low calorie intake
• Protruding abdomen & subcutaneous • Prominent ribs
fat reserved
• Poor appetite • Good appetite
• No skin changes • Pigmented & flaky skin
• Peripheral oedema present (lower • Not present
legs, lower arms, face)
• Moon facies • Old man like face
• Mild growth retardation • Severe growth retardation

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• Fructosamine (which is used for assessing long term

control in a person with hemolysis) is a glycated
Albumin

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Glucose Transporters

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SGLT-1
GLUT-2

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Cell Organelle Marker

• Plasma Membrane • Na+ K+ ATPase, 5’Nucleotidase, Adenyl Cyclase
• GolgiApparatus • Galactosyl Transferase
• Cytosol • Lactate DH
• Ribosome • rRNA
• ER • Glucose-6-Phosphatase
• Peroxisomes • Catalase
• Nucleus • DNA, DNA Polymerase
• Mitochondria • Succinate DH, Glutamate DH
• Lysosome • Acid Phosphatase
Pathways Rate limiting Enzymes

• Glycolysis • PFK-1
• TCA • Citate synthase, Isocitrate DH, Alpha KG-DH
• Gluconeogenesis • Pyr Carboxylase, PEPCK, Fructose 1,6 bisPase
• Bile Acid synthesis • 7-alpha Hydroxylase
• Niacin synthesis • QPRTase (Quinolate Phospho Ribosyl Transferase)
• Purine nucleotide synthesis • PRPP Glutamyl Amido Transferase

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Mission NEET PG Biochemistry Day - 4 by DR Smily Pruthi

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Mission NEETPG

“Your Course Completion, Our Responsibility"

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nick name of fatty acid – ‘Acyl’

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Simple Lipids Complex Lipids

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• Used in VDRL test

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 Anti-phospholipid syndrome patients

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• Phospholipids are of 2 types:

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• Sphingosine + FA + glu→ glucosyl ceramide

• Sphingosine + FA + gal → galactosyl ceramide

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• All are Autosomal recessive , Except Fabry’s disease – XR

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• Tetanus and botulinum toxins

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Q. Which of the following is not a product of complete hydrolysis of Sphingomyelin?

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Q. Which of the following processes yields arachidonic acid in mammals?

a) Elongation of stearic acid

b) Chain elongation and one desaturation of linolenic acid

c) Chain elongation and two desaturations of linoleic acid

d)Chain elongation and one desaturations of linoleic acid

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Q. C17 H31 COOH is formula of:

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Lipoprotein Another name