Enzymes are the tools of life. They do almost everything in a cell. Virtually every one of life’s chemical reactions is driven by some enzyme.
Enzymes are organic catalysts that work like a starter on a car. With a small input of energy from the starter, a car can run on its own energy all day.
Enzymes are not used up or changed when they “jumpstart” another molecule. They can perform the same task over and over.
Enzymes work very fast. They can carry out their task thousands of times per second.
Each enzyme has its own unique structural shape which allows it to bind to or “unlock” certain other molecules called substrates.
When the correct “lock” and “key” come together, a chemical bond is formed. This event may help regulate a biochemical process or it may be one step in a sequence of programmed events.
Each enzyme’s unique structural shape is encoded in DNA.
When a mutation occurs in a gene that encodes an enzyme, the structural shape of the enzyme is often changed. Thus the enzyme may not be able to “unlock” its own substrates or otherwise function properly.
A dysfunctional enzyme may lead to a dysfunctional cell.
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"Enzymes Illustrated," "Finding Enzymes,"
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What the Heck is an Enzyme?
A Kansas University professor explains biochemical catalysts using burning wood, personal
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For more about the
differences between the CPT I and CPT II enzymes, visit Fundamental
differences. For more about the
role of the CPT enzymes in mitochondrial metabolism, visit In
graphic detail.
An important point to
consider in CPT II deficiency is that the
functional enzyme is
actually made up of four subunits. If you have one normal copy of the gene and one mutant copy, or if you have a different
mutation in each of your two CPT2 genes, these subunits may come
together in many different combinations. Depending on how the defective and normal
CPT2 subunits combine, you may get varying degrees of
enzyme deficiency, resulting in variable symptoms.
--G.D. Vladutiu, Ph.D.