BIOCHEMISTRY

MODULE – 20

MADHESHIYA SAURABH
MD-1ST YEAR SEC-B

COVERAGE SUMMARY:
• Tabulate and identify the major groups of lipoproteins and
type of apolipoproteins.

There are four major groups of plasma lipoproteins

[chylomicrons, VLDT (very-low-density lipoproteins), LDL
(low-density lipoproteins), and HDL (high-density
lipoproteins)] and the protein moiety of a lipoprotein is
known as apolipoprotein or apoprotein.

LIPOPROTEINS SOURCE MAIN LIPID APOLIPOPROTEINS

COMPONENT

Chylomicrons Intestine Triacylglycerol A-1, A-2, A-4, B-48,

C-1, C-2, C-3, E
VLDL Liver (intestine) Triacylglycerol B-100, C-1, C-2, C-3
LDL VLDL Cholesterol B-100
HDL Liver, intestine, Phospholipids, A-1, A-2, A-4, C-1,
VLDL, cholesterol C-2, C-3, D, E
Chylomicrons
• Describe their functions.
Function of four major group of plasma lipoproteins:
1. Chylomicrons: chylomicrons are large triglyceride-rich
lipoproteins produced in enterocytes from dietary
lipids- namely fatty acids, and cholesterol. They are
found in the blood and lymphatic fluid where they
function to transport esterified cholesterol and
phospholipids to the peripheral tissues.
2. VLDL (very-low-density lipoproteins): VLDL carry
newly synthesized triglycerides from liver to adipose
tissue.
3. LDL (low-density lipoproteins): LDL transports
cholesterol from liver to peripheral tissues.
4. HDL (high-density lipoproteins): HDL transports
cholesterol from peripheral tissues to liver, called as
reverse cholesterol transport.
Function of apolipoproteins: apolipoproteins carry out
several roles-
1. They can form part of the structure of the
lipoprotein, for example, apo B.
2. They are enzyme cofactors, for example, C-2 for
lipoprotein lipase, A-1 for lecithin:cholesterol
acyltransferase (LCAT), or enzyme inhibitors such
as apo A-2 and apo C-3 for lipoprotein lipase, apo
C-1 for cholesteryl ester transfer protein.
3. They act as ligands for the interaction with
lipoprotein receptors in tissue, for example, apo B-
100 and apo E for the LDL receptor and apo E for
the LDL-receptor-related protein-1 (LRP-1).
• In a diagram, trace the transport of triglycerides from the
intestine to liver and liver to extrahepatic tissues.
• Identify and briefly describe 3 sphingolipid disorders.
1. Farber disease: it is an autosomal-recessively
inherited, lysosomal storage disorder caused by acid
ceramidase deficiency and associated with distinct
clinical phenotypes. Children with significant
neurological involvement usually dies early in infancy,
whereas patients without or mild neurological finding
suffers from progressive joint deformation and
contractures, subcutaneous nodules, inflammatory,
periarticular granulomas, a hoarse voice and finally
respiratory insufficiency caused by granuloma
formation in the respiratory tract and interstitial
pneumonitis leading to death in the third or fourth
decade of live.
2. Niemann-Pick disease: it is a subgroup of lipid storage
disorders called sphingolipidoses in which harmful
quantities of fatty substances, or lipid, accumulate in
the spleen, liver, lungs, bone marrow, and brain. In
the classic infantile type-A variant, a missense
mutation causes complete deficiency of enzyme
sphingomyelinase. Clinical symptoms associated with
this disease are enlarged liver and spleen, mental
retardation, fatal in early life.
3. Tay-Sachs disease: it is a rare genetic disorder passed
from parents to child. It is caused by the deficiency of
an enzyme Hexosaminidase that helps break down
fatty substances. These fatty substances called
gangliosides, which accumulation to toxic levels
results in the destruction of nerve cells in the brain
and spinal cord. The most common form is infantile
Tay-Sachs disease which became apparent around
three to six months of age, with baby losing the ability
to turn over, sit, or crawl. Clinical symptoms are
mental retardation, blindness, muscular weakness.