Neuronal ceroid lipofuscinosis
| Neuronal ceroid lipofuscinosis | |
|---|---|
| Other names | NCL |
| Confocal image of a spinal motor neuron showing stained lipofuscin granules in blue and yellow. | |
| Specialty | Endocrinology |
Neuronal ceroid lipofuscinosis is a family of at least eight genetically separate neurodegenerative lysosomal storage diseases that result from excessive accumulation of lipopigments (lipofuscin) in the body's tissues.[1] These lipopigments are made up of fats and proteins. Their name comes from the word stem "lipo-", which is a variation on lipid, and from the term "pigment", used because the substances take on a greenish-yellow color when viewed under an ultraviolet light microscope. These lipofuscin materials build up in neuronal cells and many organs, including the liver, spleen, myocardium, and kidneys.
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