A cell is the smallest structural unit of the body. Each cell acts like its own tiny, self-contained organism, performing such functions as reproduction, consumption, and waste disposal. These activities are carried out by smaller components within the cell called organelles. The nucleus (plural: nuclei) is the organelle that functions as the cell's "command center," and it contains all of a person's genetic material. The organelles most relevant to lysosomal storage disorders are lysosomes, which are responsible for breaking down waste materials in cells.

Lysosomes (in yellow) break down waste in cells

Cells are composed of many different types of molecules, of which the most important are proteins. The human body contains tens of thousands of proteins, which make up more of a cell's mass than anything else except water. Some proteins are highly specialized to control specific activities.

Enzymes are a category of proteins responsible for speeding up chemical reactions to help regulate the body's development and function--for example, converting food into energy or breaking down waste substances in the lysosomes.

We need enzymes to live and function properly--without them, certain bodily operations can go wrong, causing illness or even death. But just how does the body know how to produce the necessary enzymes in the right amounts? That's encoded in our genetic material in the cell nucleus.

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