We are well aware of the warning that ‘Cholesterol is BAD
for us’. Our body synthesizes about 80
percent of this much maligned fat from simpler molecules in a complex 37 step process.
Cholesterol itself is but an intermediate substance needed by our body to
synthesize critical substances needed for our well-being and survival. Experiments
with mutant cell lines and specific inhibitors of cholesterol biosynthesis
suggested that is essential for the growth of mammalian cells. With little or
no cholesterol, the fetus will be aborted or has serious defects.
What could happen if our body could
not use cholesterol?
SLOS or 7-dehydrocholesterol reductase deficiency is an inborn error of
cholesterol synthesis. Children born with this genetic disorder have problems
ranging from intellectual disability and behavioral problems to life-threatening
malformations. The condition includes defects in the brain, face, limbs, kidneys,
liver, heart and genital area in varying degree of severity.
People with Cerebrotendinous
xanthomatosis lack an enzyme to convert cholesterol into bile acids
normally. The function of bile acids is to emulsify lipids (fat) and
fat-soluble vitamins to enable our body to absorb and use them. Children with
this disorder experience diarrhea, cataracts and tendon xanthomas, which are
soft bumps under the skin made up of yellow fatty deposits. These fatty
deposits accumulate in the blood, nerve cells and the brain.
As they grow older, these children may lose mental capability and begin to show
signs of abnormal movements, cholesterol deposit in the brain, Degeneration of
parts of the brain , Psychiatric symptoms, Seizure and Premature atherosclerosis (hardening of blood
vessel).
Cystic fibrosis or cholestatic
liver disease is a disorder where sufferers have an impaired capacity to
absorb dietary fat and therefore fat-soluble vitamins as well. They experienced
injury to the sensory nerves (peripheral neuropathy), impaired balance and
coordination (ataxia), muscle weakness (myopathy), and damage to the retina of
the eye (pigmented retinopathy).
The medical profession recognizes eight different inherited
disorders of cholesterol biosynthesis that lead to congenital abnormalities.
These diseases showed a decrease in the concentration of cholesterol in the
body can result in severe health problems. The next article will focus on the
numerous vital roles cholesterol plays that ensure our well-being.
