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More information about protein and
isoleucine
Protein is an important part of food. It is needed by our bodies
to build muscles and to keep the body functioning. In children it is
essential to help them grow.
Different foods have different amounts of protein.
These foods are high in protein: beef, pork, chicken, poultry, fish,
and milk and cheese. Foods that have some protein are vegetables,
rice, corn, bread and cereals. Fruit, sweets and oils are very low
in protein.
Protein can be thought of as long chains made up of smaller units,
like beads on a string make up a necklace. The small units are
called amino acids. There are 20 different amino acids that make up
proteins. One of the 20 amino acids is called isoleucine. Foods that
are high in protein are high in isoleucine.
| The symbol for isoleucine in the protein below is:
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Protein is a long chain of amino acids, like beads on a string.
When protein is eaten, our bodies break it apart into the different
amino acids.
The amino acids, including isoleucine, are used to build new
proteins that are used by the body.
Typically, any isoleucine that is not used to make new proteins is
broken down to make energy. The body takes several steps to change
isoleucine to energy.
Extra isoleucine is broken down to make energy.
People who have 2-MBADD use the amino acid isoleucine from their
food to build new proteins, just like anyone else. However, after
the body is finished using the isoleucine it needs for building new
proteins, extra isoleucine is not broken down to make energy.
Someone with 2-MBADD cannot make energy from extra isoleucine. There
is a block at one of the steps the body takes to break down the
isoleucine. Since the body cannot break down isoleucine as usual, it
makes substances, called organic acids, which can be harmful to the
body.
In someone who has 2-MBADD
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