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Tay-Sachs, Canavan, GM1 and Sandhoff diseases


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Term Definition
N-acetyl-a-acid (NAA)

Aspartoacylase breaks down NAA into aspartic acid (an amino acid used in making proteins) and acetic acid. The cycle of production and breakdown of NAA appears to be critical for maintaining the brain's white matter, which consists of nerve fibers covered by myelin. NAA is likely essential for making certain fats (lipids) that are used to produce myelin.

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Neuronal stem cells

A stem cell found in adult neural tissue that can give rise to neurons and glial (supporting) cells. Examples of glial cells include astrocytes and oligodenrocytes. Definition: National Institute of Health (http://stemcells.nih.gov/info/glossary.asp)

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Nerve cells, the structural and functional unit of the nervous system. A neuron consists of a cell body and its processes- an axon and one or more dendrites. Neurons function by starting and conducting impulses. Neurons transmit impulses to other neurons or cells by releasing neurotransmitters at synapses. Definition: National Institute of Health (http://stemcells.nih.gov/info/glossary.asp)

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